Lesions of the Sellar Region Which May Resemble Macroadenomas Lesiones De La Región Selar Que Pueden Parecerse a Macroadenomas

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ORIGINAL ARTICLE

Lesions of the sellar region which may resemble macroadenomas Lesiones de la región selar que pueden parecerse a macroadenomas

Stelios Cedi-Zamudio1, M. Gray-Lugo1, A.E. Vega-Gutiérrez2, V.H. Ramos-Pacheco3, L. Manola-Aguilar4 and G.M. Guerrero-Avendaño5 1Médico Residente del Servicio de Radiología e Imagen; 2Medico Radiólogo especialista en Resonancia Magnética; 3Médico Residente de Curso de Alta Especialidad en el servicio de Resonancia Magnética; 4Médico Residente del Servicio de Neuropatología; 5Medico Radiólogo Intervencionista. Hospital General de México Dr. Eduardo Liceaga, Ciudad de México, México

ABSTRACT

Correspondence to: Stelios Cedi-Zamudio Médico Residente del Servicio de Radiología e Imagen Hospital General de México Dr. Eduardo Liceaga Ciudad de México, México Received in original form: 22-07-2016 E-mail: [email protected] Accepted in final form: 17-09-2016 1665-2118/©2018 Sociedad Mexicana de Radiologia e Imagen, AC. Publicado por Permanyer México SA de CV. Este es un artículo Open Access bajo la licencia CC BY-NC-ND (http://creativecommons.org/licenses/by-nc-nd/4.0/). Anales de Radiología México. 2016;15

INTRODUCTION are headache, endocrinological disturbances that can be associated to hypopituitarism, hy- The study of the sellar region has had import- perprolactinemia, hypersecretion of growth ant changes throughout time with the evolu- hormone, pituitary apoplexy, and III, IV, and tion of imaging methods that have growingly VI cranial nerve disorders with optic neurop- improved the resolution of images and have athy leading to diplopia with cavernous sinus displaced diagnostic studies used in the 70s syndrome.1 and 80s last century, such as angiography or pneumoencephalography as the first choice The use of MRI as a standard method pro- methods for diagnosis; computed tomogra- vides more information about disorders in phy and magnetic resonance imaging (MRI) the sellar region. have grown in preference1.

The main occupational lesion of the sellar re- Objective gion is a tumor, and pituitary adenoma is the most common in adult age, followed by cra- Learn the different differential diagnoses, niopharyngioma. Nearly 10% of patients have besides, pituitary macroadenoma that in- differential diagnoses that are a diagnostic volves the sellar region and that can be clin- and therapeutic challenge. Learning about ically similar to the most commonly reported these conditions by their imaging character- pituitary diseases, and look at the imaging istics and their histopathological correlation characteristics with their histopathological will bring more information to give a timely correlation at the Hospital General de México diagnosis and information of the disease that Dr. Eduardo Liceaga. may be similar to what is most commonly reported2,3. MATERIALS AND METHODS In a retrospective study conducted in Pennsyl- vania, where 131 cases with involvement of the Retrospective, descriptive study in patients sellar and parasellar region were analyzed, the with a diagnosis of sellar region tumor ad- most common lesions were found to be mac- mitted to the Hospital General de México roadenomas, craniopharyngiomas, meningio- Dr. Eduardo Liceaga. The database of the MRI mas, and aneurysms2. Investigation of tumors department was reviewed from March 2011 of the sellar region may be difficult due to the to March 2016. Of a total of 5397 MRI scans, many variable lesions found and, also, because we found 117 patients with diagnosis of le- of their different imaging characteristics. Ac- sions in the sellar region. The type of tumor, cording to prevalence, they are reported in gender, and age of patients was reported, as order of frequency as macroadenoma, menin- well as the definitive diagnosis, which was gioma, craniopharyngioma, and aneurysms2,3. made after a surgical procedure to remove the tumor or take a biopsy, and after getting The clinical manifestations depend on the ex- the histopathological diagnosis. Tumors of tension of the disease, and main symptoms the sellar region are comprised trying to

252 S. Cedi-Zamudio, et al.: Lesions of the Sellar Region

describe their main features in order of fre- evaluated, the features that point to the diag- quency and the clinical characteristics found nosis must be identified: location of the le- at Hospital General de México. sion, morphology, behavior after contrast ad- ministration, and number of lesions.

RESULTS Location A greater involvement of the sellar region was found in female patients (64%) in the Sellar region fourth decade of life, and the main tumor was craniopharyngioma (49.1%) followed by me- We need to determine where the lesion ningioma (23.2%); on imaging studies, they originated, if it is from the pituitary gland are identified as lesions of mixed appearance altering its morphology and leading to re- whose most common vector of growth is su- modeling of the sellar region, we must always prasellar. Imaging agreement was 61.2% suspect the most common tumor, which is a Table 1. The histopathological diagnosis of macroadenoma. Sometimes, tumors that de- macroadenoma was excluded for the analysis. pend on the infundibulum have a vector of growth to the sellar region, with displace- ment of the pituitary gland and compression Histological diagnosis over the ventral surface of the sellar region such as the pilocytic astrocytoma (Fig. 1). This Biopsies were taken using a transsphenoidal is a slow-growing tumor. It accounts for 5-6% approach. Diagnoses other than tumors were of all the gliomas, with the highest incidence found mostly to be one case of histiocytosis, in the second decade of life, with no gender tuberculoma, macroadenoma with lympho- prevalence. On MRI, it is ISO or hypointense cytic hypophysitis, and cystic lesions (epider- on TI and hyperintense on T2 with mixed moid, neuroepithelial). component (cystic area with a mural nodule that enhances after contrast); vasogenic edema is uncommon4,5. If the lesion is located in DISCUSSION the posterior pituitary, it must be considered a pituicytoma. Pituitary macroadenoma is the most common lesion of the sellar region. Patients with a lesion in the sellar region must always be ap- Anterior region proached by getting information about the age of presentation, gender, and clinical If the lesion is located anterior to the sellar picture that may indicate a fast course, prob- region, there are differential diagnoses such able malignant etiology, or immune diseases as glioma of the optic nerve; it is associated that could make us think of an opportunistic with neurofibromatosis Type I and it accounts infection or, in our setting, tuberculosis. for 10-15% of supratentorial tumors, they are Whenever a tumor of the sellar region is more common in pediatric age. On MRI, they

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Table 1. Histological diagnosis and rates of presentation in the population of the Hospital General de México Dr. Eduardo Liceaga, from 2011 to 2016. Patients with a diagnosis of pituitary macroadenoma were excluded. Histological Number of Rate Age Females Males Size Growth Component Bleeding/ Agreement diagnosis cases (decade) calcifications with radiological diagnosis

Craniopharyngioma 57 49.1 2ª y 3ª 33 24 3.0 cm IS, SS Mixed Bleeding, 39 calcifications

Meningioma 27 23.2 4ª 22 5 3.8 cm IS, SS, PS Mixed NP 21

Chordoma 9 7.7 4ª y 5ª 5 4 5.4 cm Clivus, Solid Calcifications 5 sphenoidal

Germinoma 7 6.0 2ª y 3ª 6 1 2.7 cm IS, PS Mixed NP 2

Plasmocytoma 5 4.3 6ª 2 3 5.0 cm Clivus, Solid Calcifications 2 sphenoidal

Pilocytic strocitoma 4 3.4 1ª 2 2 7.7 cm IS, SS Mixed Bleeding 0

Chondrosarcoma 2 1.7 5ª 1 1 2.7 cm IS, PS, clivus Solid Calcifications 2

Histiocytosis 1 0.8 3ª 1 0 2.8 cm IS, SS Solid NP 0

Epidermoid cyst 1 0.8 4ª 0 1 2.5 cm IS Mixed NP 0

Neuroepithelial cyst 1 0.8 4ª 0 1 2.4 cm IS Mixed NP 0

Metastasis 1 0.8 8ª 1 0 3.0 cm IS Mixed Bleeding 0

Tuberculoma 1 0.8 4ª 1 0 3.0 cm IS, PS Mixed NP 0

Lymphocytic 1 0.8 4ª 1 0 3.0 cm IS, PS Solid Bleeding 0 hypophysitis

Total number of 117 75 42 61.2% patients

Mixed component: solid and cystic content; size: longitudinal diameter in the longest axis; Growth: IS: intrasellar; SS: suprasellar extension; PS: parasellar extension; NP: not present. are hypointense on T1, hyperintense on T2; para/suprasellar region2. On MRI, they are morphologically they tend to have a mixed isointense to grey matter on T1 and T2; some component with cystic areas, the solid com- may have calcifications, a cystic and hemor- ponent enhances with contrast. rhagic component; peritumoral edema is associated. There is strong and fast enhancement after contrast with the dural tail sign (Fig. 2).1,2,6 Parasellar region

Lesions located in the parasellar region may Posterior region have a vascular etiology (aneurysm), meningioma, or schwannoma. Meningiomas account In tumors located in the posterior region of for 18% of intracranial tumors located in the the clivus causing expansive lytic lesions with parasellar and suprasellar region; the largest suprasellar and retrosellar growth, a chondro- ones may originate from the sphenoidal plane ma must be considered, which is a locally ag- or the sphenoid wing with extension to the gressive tumor, accounting for 1% of

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A B C

Figure 1. (A) Infantile desmoplastic astrocytoma, magnetic resonance imaging, axial plane, T2 weighted with a predominantly hypointense lesion in the sellar region, with a hyperintense area associated with bleeding and parasellar and clival vector of growth. (B) Sagittal plane, mixed component with cystic areas. (C) T1-weighted sagittal plane with contrast, moderate enhancement of the solid component.

A B C

Figure 2. (A) Magnetic resonance imaging, T1-weighted axial plane with contrast: a meningioma is observed located in the sellar region, with lobulated shape, with a sellar, right parasellar vector of growth, with significant enhancement after contrast. (B) T1-weighted coronal plane with contrast, suprasellar, and right parasellar vector of growth, with solid component. (C) T2-weighted sagittal plane with clival vector of growth compressing the pons and hypothalamus. intracranial tumors and 4% of primary bone bleedings, and protein content are hypointense tumors, with prevalence in the fourth decade on T2 (Fig. 3). Chondrosarcoma accounts for of life with 2–1 male-to-female ratio. Its origin 0.2% of intracranial tumors; it originates from is in remnants of the primitive notochord. pluripotent cells or from persistence of carti- 50% are located in the sacrococcygeal region, laginous remnants after endochondral ossifi- 35% intracranial, and 15% in the vertebral cation, located in the midline of the skull base bodies. They are divided into typical and in the petroclival synchondrosis. It is found in chondroid3,6,7. On T1 sequence, they are hy- the fourth decade of life with female predom- pointense and hyperintense on T2, they may inance; it is associated to Ollier and Maffucci have bleedings that can be seen in the echo syndromes (Fig. 4)8,9. Plasmacytoma is a rare gradient sequence. Areas with calcifications, tumor with involvement of the skull base

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A B C

Figure 3. (A) Magnetic resonance imaging, T2-weighted coronal plane: chordoma located in the clival region with intrasellar and retroclival vector of growth of the left predominance, lobulated shape with predominantly hyperintense mixed component with destruction of the clivus. (B) T2-weighted sagittal plane with destruction of the clivus. (C) T1-weighted sagittal plane with contrast, moderate enhancement, and compression of the pons.

A B C

Figure 4. (A) Skull computed tomography scan, axial plane: chondrosarcoma in the clival region that destroys it and the greater sphenoid wing on the left side. (B) Sagittal plane: significant distortion of the clival region with calcifications of diffuse distribution. (C) Sagittal plane with contrast: moderate enhancement of the solid component located in the clival region.

(leptomeninges). It originates from plasma component is mixed with multiple areas of cells, and it can present as a solitary lesion cystic or solid predominance we will suspect (plasmacytoma) or multiple lesions (multiple the second most common lesion in our pop- myeloma). On MRI, it is hypointense on T1 ulation: craniopharyngioma; a tumor located and isointense on T2 with no enhancement in the sellar/suprasellar region most com- after contrast. The age of onset is the fifth mon in pediatric age (6–9% of tumors of the decade of life and it is more common in wom- central nervous system4) with a second peak en as plasmacytoma and more common in in the fourth and fifth decade of life. They males as multiple myeloma10. grow from Rathke’s pouch11. Two subtypes have been recognized: adamantinomatous Morphologic component (mixed pattern made of cystic and solid areas with predominance of cystic regions with en- The predominant component of lesions is hancement of the solid component after con- important to point to the diagnosis, if the trast and with calcifications), the cystic

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A B C

Figure 5. (A) Magnetic resonance imaging, FLAIR weighted axial plane: adamantinomatous craniopharyngioma in the sellar region, with hyperintense ovoid shape and mild perilesional edema. (B) T2-weighted coronal plane: cystic lesion in the sellar region and suprasellar vector of growth with compression of the hypothalamus. (C) T2-weighted sagittal plane: cystic lesion in the sellar region and suprasellar vector of growth with compression of the hypothalamus.

A B C

Figure 6. (A) Magnetic resonance imaging, T1-weighted sagittal plane: sellar germinoma with lobulated shape, solid component isointense to brain parenchyma, with suprasellar vector of growth. (B) T1-weighted coronal plane with contrast: significant enhancement after contrast. (C) T1-weighted sagittal plane with contrast: significant enhancement after contrast with suprasellar vector of growth. component can appear hyperintense on T1 distribution (dysgerminoma, teratoma, and due to the high-protein content, the papillary craniopharyngioma). type has a major solid component (Fig. 5)6,12. If the component has fat content, it points to dermoid cysts that are heterogeneous cystic Behavior after contrast inclusions containing fat, calcifications, and odontogenic tissue. The sellar and parasellar The use of contrast for evaluation of lesions is region are most commonly involved, on MRI, important because it helps to better outline they are hyperintense on T1 and T26. Asso- the extension of the lesion and to determine if ciated findings must be evaluated, such as the carotid arteries or the basilar artery are bleedings or calcifications, and if they are encased, what neighboring structures are distributed toward the periphery (chondro- compromised, and the type of enhancement, ma, sarcoma) if they are diffuse in if it is homogeneous or heterogeneous.

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A B C

Figure 7. (A) Magnetic resonance imaging, sagittal plane: histiocytosis in the sellar region with increased size, predominantly isointense to brain parenchyma with suprasellar vector of growth. (B) T2-weighted sagittal plane with a lesion in the sellar region with increased size, predominantly isointense to brain parenchyma, with some areas of cystic appearance, suprasellar vector of growth and compression of the optic chiasm. (C) T1-weighted sagittal plane with contrast: moderate ring enhancement of the lesion and solid component in the central region.

A B C

Figure 8. (A) Magnetic resonance imaging, T2-weighted sagittal plane: macroadenoma with lymphocytic hypophysitis in the sellar region, increased size, isointense to brain parenchyma with suprasellar vector of growth. (B) T2-weighted coronal plane with predominantly cystic lesion, with some hyperintense areas associated with bleeding, suprasellar and parasellar vector of growth of the right predominance causing compression of the optic chiasm. (C) T1-weighted sagittal plane with contrast: mild enhancement of the solid component and more toward the periphery with ring shape.

tumors with male sex predominance. They Number of lesions are located in the suprasellar and pineal region, but there are cases reported in the The presence of two or more lesions must brainstem and the basal ganglia13. The make us suspect metastatic disease, and peak incidence is during adolescence. Of- if lesions are in the midline and located in other sites besides the sellar region, ten, the type of expansion involves the such as the pineal gland, we must consid- ventricular system and the subarachnoid er a germinoma. Germinoma is a tumor space, capable of infiltrating the adjacent that originates in pluripotent cells ac- soft tissue and bone structures (Fig. 6)13. counting for 0.4-3.4% of intracranial The lesions to keep in mind (briefly

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