Differential Diagnosis of Sellar Lesions
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1 Differential Diagnosis of Sellar Lesions Manish K. Aghi, M.D., Ph.D. Assistant Professor California Center for Pituitary Disorders Department of Neurosurgery University of California, San Francisco (UCSF) Page 1
2 Introduction - Sellar Anatomy SUPERIOR ANTERIOR POSTERIOR INFERIOR Page 2
3 Sellar anatomy on MRI Pituitary gland boundaries: Superior pituitary stalk (deviates away from tumors), optic chiasm (pushed up by macroadenomas) Inferior sphenoid sinus, sellar bone Right/left cavernous sinus/carotid arteries Page 3
4 Differential Diagnosis of Sellar Masses 1. Cystic Lesions Rathke s cleft cyst, Craniopharyngioma, Arachnoid Cyst 2. Other Neoplasms Meningioma,, Germ Cell Tumor, Chordoma,, Granular Cell Tumor, Pituicytoma,, Glioma, Metastases, Lymphoma 3. Inflammation/Infection Sarcoidosis,, Tuberculosis, Pituitary Abscess, Langerhans Histiocytosis,, Lymphocytic Hypophysitis 4. Empty sella syndrome 5. Pituitary Adenomas (91%) Page 4
5 Case 34 yo F presents with headache, that eventually led her primary care physician to get a brain MRI. T1 T1 gado Prolactin 55 ng/ml, other hormones within normal limits. T2 Page 5
6 Rathke s cleft cyst Originate between anterior and posterior lobe from embryologic Rathke s pouch. Anterior lobe forms from anterior wall of Rathke s pouch and pars intermedia forms from posterior wall of Rathke s pouch. Symptoms headache most common; visual disturbance or hypopituitarism less common Page 6
7 Rathke s Cleft Cyst - MRI Differential diagnosis is usually cystic adenoma vs Rathke s cleft cyst, Rathke s cleft cyst defined by: Stalk typically midline abnormality between anterior and posterior lobe 1. Two types: i. T1 dark, T2 bright (one third) fluid like CSF ii. T1 bright, T2 variable (two third) fluid is mucus, higher rate of presenting with h/a (100%) or gland dysfunction (40%); higher rate of postop diabetes insipidus than adenomas Page 7
8 Rathke s Cleft Cyst Treatment 1. transsphenoidal surgery if larger than 1 cm or clearly symptomatic; need to drain cyst and remove as much wall as possible 2. 20% recur within first 2 years 3. rate of headache improvement 60-80% 4. rate of hormone dysfunction improvement 20% Page 8
9 Rathke s Cleft Cyst Cyst wall typically has columnar or cuboidal epithelium, but squamous metaplasia can occur and increases risk of recurrence by as much as 3-3 to 4-fold4 Page 9
10 Pituitary Arachnoid Cyst Congenital or acquired; herniation of arachnoid membrane through incompetent diaphragm or arises from arachnoid rests below the diaphgragm Symptoms 90% have headaches, 35% have visual field defect, 35% have hypopituitarism MRI similar to Rathke s cleft cyst containing watery contents rather than mucus, T2 bright T1 dark Treatment transsphenoidal drainage and wall biopsy, headache gets better but endocrine function improvement worse than with other cysts. 20% recurrence at 10 years. Page 10
11 Craniopharyngioma Age 5 to 10 Histologically benign looking tumor (developing from nests of epithelium derived from Rathke s pouch) with malignant behavior due to invasion of surrounding structures and recurrence after gross total resection. Single large or multiple cysts filled with turbid, proteinaceous brown-yellow material that sparkles due to cholesterol crystals. Usually start sellar, can have suprasellar extension with invasion into hypothalamus. Page 11
12 Craniopharyngioma Radiation used for residual tumor after surgery, although hypopituitarism and other side effects in the pediatric population will ensue. 10-year recurrence rate 83% for tumors larger than 5 cm, 20% for tumors smaller than 5 cm Page 12
13 Overview of cystic pituitary lesions Cranio Rathke Arach Psychiatric disturbances unique to craniopharyngioma, get better with surgery Arachnoid cyst patients present older (fifth decade of life) Endocrine dysfunction rare in arachnoid cyst, if present in arachnoid cyst won t get better with surgery Source: JCEM 84: 3972, 1999 Page 13
14 Cystic Pituitary Lesions - Recurrence Arachnoid cysts 20% recur after 10 years; Rathke s 20% recur within first year or 2; Craniopharyngioma most recur, spread over 10 years Source: JCEM 84: 3972, 1999 Page 14
15 Case 33 year old female with severe headaches, n/v,, weight loss, hyperprolactinemia, hypoadrenalism, hypothyroidsm. MRI with intrinsically T1 bright, T2 intermediate signal mm lesion in left inferoposterior sella Page 15
16 Pituicytoma New diagnosis created by WHO in 2007 WHO grade I tumor of the pituicytes,, which are stromal (supporting) cells of the posterior lobe or stalk. Present with visual field defect, or asymptomatic incidental finding on MRI More vascular than pituitary adenoma and tough to access (posterior lobe, sometimes stalk) so surgery risky. Page 16
17 Pituicytoma Five cases at UCSF Mean age 53 (range 33-71) 3 men, 2 women 1 incidental finding; 2 panhypopituarism,, 1 visual symptoms; 2 with over 20 lb weight loss Page 17
18 Pituicytoma GFAP+ MIB % No hormones, no neurosecretory granules Well circumscribed Low recurrence rate but readily symptomatic due to location Page 18
19 Metastasis to the Pituitary Gland 1% of sellar lesions are mets - Breast, Lung most common Posterior Lobe more common than anterior lobe (direct blood supply), also can grow along stalk Patients years younger than average brain met patient Posterior lobe mets often present with diabetes insipidus and are often treated with radiosurgery Page 19
20 Case 33 year old male presents with right eye sealed shut for 10 days and several months of abdominal pain. Exam R 3 rd nerve palsy, but acuity intact. Hormone function reveals low testosterone, low thyroid hormone. T1 gado coronal T1 sag gado Page 20
21 Pituitary Lymphoma MRI isointense to brain on T1 and T2, enhance homogeneously after gado (pituitary adenoma is hypointense on T1, hyperintense on T2, and have delayed gado enhancement compared to normal gland) Treatment transsphenoidal biopsy then chemotherapy Page 21
22 Pituitary Germinoma Most common causes of central DI in children/adolescents are Langerhans histiocytosis and germinoma. MRI thick homogeneously enhancing stalk; sometimes associated with pineal lesion. Treatment Transsphenoidal biopsy, then radiation to 40 Gy, 90% long-term survival Page 22
23 Case 24 yo F with intermittent h/a for 6 months, 1 month of amenorrhea, 2 weeks of polyuria/polydipsia.. No other sx.. Sp grav 1.005, daily urine output 7 L, decreased estradiol,, low T3/T4, prolactin 25 ng/ml, low GH, ESR 14, WBC 6.2 Page 23
24 Pituitary Abscess Can be primary (in normal gland) or secondary (in pre- existing pathology like adenoma or Rathke s cleft cyst). Presenting symptom hypopituitarism, headache, visual field cut. Fever and meningismus are rare. Source of infection unclear hematogenous versus local spread from sphenoid sinus both speculated Page 24
25 Pituitary Abscess MRI - cystic lesion with heterogeneous signal and peripheral enhancement causing hypopituitarism.. Restricted diffusion highly predictive when present. Only 50% grow out organisms, when they do gram + and gram equally common, fungi less common. Page 25
26 Culture Negative May Still Benefit from Antibiotic Treatment Culture negative infected Rathke s cysts recur more frequently than culture positive cysts treated with antibiotics; antibiotic treatment restores the recurrence rate to that of noncultured/noninfected cysts Percent recurrence NO ANTIBIOTIC CULTURE-NEGATIVE CULTURED ANTIBIOTIC-TREATED NONCULTURED Months Page 26
27 Same case continued One year later all hormone function except GH has resolved and MRI shows: Page 27
28 Empty sella syndrome Sella filled with CSF causing pituitary gland to be flattened against floor of sella.. In these patients, the diaphragm sellae is limited to a thin rim of tissue around a huge infundibular foramen. Much more common in females than males. Seen in 6% of autopsies. 30% of empty sella patients are hypertensive. Can be primary or secondary to burned out pituitary tumors after apoplexy, post-radiation Headache most common symptom. 30% GH deficient. Page 28
29 Case 28 yo F with severe h/a 4 days after normal vaginal delivery. Labs reveal low prolactin 0.5 ng/ml (normal 0-20) 0 and low ACTH Page 29
30 Another case 39 year old male with 4 weeks of h/a, n/v,, and left eye visual loss To OR at another institution where left carotid injury occurred Page 30
31 Another case (continued) Transferred to our institution for carotid injury/pseudoaneurysm management (stenting( of left supraclinoid ICA) On coumadin post-stent stent and on replacement steroid therapy. 3 month follow-up imaging Page 31
32 Lymphocytic Hypophysitis Lymphocytic pituitary infiltration during or after pregnancy Symptoms present with ACTH deficiency (whereas nonfunctional adenoma presents with GH or FSH/LH deficiency), 1/3 get hyperprolactinemia (stalk effect) 2/3 get hypoprolactinemia (gland compression) MRI anterior gland enhancement that may extend to stalk Diagnosed by transsphenoidal endonasal pituitary biopsy Treated with steroids 3 weeks steroids Page 32
33 Langerhans Histiocytosis In pediatric/adolescent patients, most common causes of DI are Langerhans histiocytosis and germinoma. MRI thick stalk, posterior lobe loses its T1 brightness but now enhances. Need biopsy to differentiate from germinoma. Treat with alkylating agent + steroid. Prognosis good. Page 33
34 Pituitary Sarcoidosis 5% of sarcoid cases have CNS involvement, but hypothalamus, pituitary stalk, and pituitary are most common sites of CNS involvement. DI is most common presenting symptom, followed by anterior pituitary hormone deficiency MRI with enhancing fullness of posterior gland. Treatment transsphenoidal biopsy, treat with 40 mg prednisone daily, lesion will likely resolve, but patient will likely continue to need ddavp if presented with DI. Page 34
35 Case 35 yo F with galactorrhea and amenorrhea. Labs reveal prolactin 43 ng/ml (normal 0-20), 0 TSH 283 µu/ml (normal 0-3). 0 Page 35
36 Pituitary Hyperplasia Sx high prolactin,, high TSH but prolactin symptoms predominate Cause hypothyroidism, TRH drives prolactin release Treatment thyroid hormone Path hyperplasia of thyrotrophs and lactotrophs. 4 months synthroid Page 36
37 Pituitary Adenoma - Nonsecretory Radiographic features: abnormality in anterior lobe of gland usually anteroinferior aspect of gland; tend to be on one side and push stalk to opposite side; most are somewhat T2 bright indicating softness, fibrous adenomas can be T2 dark; characteristic T1 enhancement pattern causing adenoma to appear darker than adjacent gland. Page 37
38 Pituitary Adenoma - Nonsecretory Stalk effect increased prolactin due to tumor size, increased adenoma size up to 3 cm makes a higher prolactin attributable to stalk effect. Page 38
39 Pituitary Adenoma - Nonsecretory Pathologist should stain tumors for hormones, especially ACTH, because silent corticotrophic adenomas may have higher risk of recurrence and risk of delayed Cushing s disease. Page 39
40 Page 40
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