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Table 1 Previous reports of sellar involvement in GPA

From: Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

1st Author & year PMID No. of cases Age at GPA diagnosis Age at time of sellar involvement Sex Pituitary Imaging (MRI unless stated) Endocrinological abnormalities Non-CNS features ANCA
Haynes 1978 [29] 692550 1 26* 25 M NA DI Hyperprolactinaemia Normal TSH
Normal LH & FSH
Lung, renal N/A
Hurst 1983 [30] 6625709 1 47* 47 F CT normal DI Normal prolactin Normal TSH
Post-menopausal LH/FSH
Polyarthritis, ENT, ocular, mucocutaneous, pulmonary N/A
Lohr 1988 [31] 3172100 1 19* 19 F Intrasellar mass N/A ENT, pulmonary N/A
Rosete 1991 [32] 1865428 1 51* 51 F CT: pituitary enlargement DI N/A N/A
Czarnecki 1995 [33] 7611087 1 31 34 F Sellar mass with suprasellar extension. Enhancement of the stalk and hypothalamus. Loss of PS. DI Hyperprolactinaemia ENT, arthralgia N/A
Roberts 1995 [34] 7758239 2 71* 71 F Intrasellar mass with suprasellar extension DI (post-surgery)
↓ TSH
↓ LH & FSH
↓ cortisol
Normal prolactin
None cANCA +ve
28* 28 F Intrasellar mass with low-density centre DI (post-surgery) Normal prolactin Normal TSH
Normal LH & FSH
Normal cortisol
Ocular, arthralgia, cutaneous, renal cANCA +ve
Bertken 1997 [35] 9265867 1 36* 36 F Macrocystic pituitary mass with suprasellar extension. Hydrocephalus DI (post surgery) ↓ TSH response ↓ LH & FSH responses ENT, pulmonary, ocular ANCA -ve
Hajj-Ali 1999 [36] 10461488 1 N/A 21 F Normal DI N/A N/A
Katzman 1999 [21] 10219422 2 41* 41 F Pituitary enlargement. Loss of PS DI Hyperprolactinaemia Normal TSH Normal LH & FSH Constitutional, arthralgia, ENT, ocular, cutaneous cANCA +ve
18* 18 F Pituitary enlargement with contrast-enhancement.
Loss of PS
DI
Hyperprolactinaemia
Normal TSH
Normal LH & FSH
ENT, pulmonary cANCA +ve
Miesen 1999 [37] 10069203 1 46* 45 M Stalk thickening, contrast-enhancement.
Loss of PS
DI
Hyperprolactinaemia
Hypogonadism
Normal TSH
Normal LH & FSH
Normal cortisol & ACTH
ENT, renal, pulmonary ANCA +ve
Goyal 2000 [38] 11003280 1 N/A (many years before pituitary involvement) 48 F Sellar mass with suprasellar extension. Contrast-enhancement DI
↓ TSH
Renal, pulmonary cANCA +ve
Tappouni 2000 [39] 11096156 1 58* 57 F Pituitary mass DI Constitutional, ENT, cutaneous, renal, pulmonary PR3 +ve
Woywodt 2000 [40] 11028850 1 30* 30 M N/A (diagnosed at autopsy) N/A N/A N/A
Garovic 2001 [41] 11136194 1 47* 47 F Cystic enlargement of the pituitary.
Non-enhancing with gadolinium
DI
↓ prolactin
↓ TSH
↓ gonadotropins
Constitutional, cutaneous, pulmonary cANCA +ve
Tao 2003 [42] 14642162 1 N/A 19 F Pituitary and stalk enlargement with heterogenous enhancement DI
↓ TSH
Hypogonadism
N/A N/A
Muir 2004 [43] 15150009 1 13* 13 M Diffuse pituitary enlargement.
Foci of ↑ T1 signal. More extensive ↑ T2 signal. Central contrast-enhancement.
Loss of PS.
DI ENT, pulmonary ANCA +ve
Vittaz 2004 [44] 15687906 2 45 47 M Pituitary mass, with contrast-enhancement.
Pituitary stalk thickened & infiltrated.
Loss of PS
Hyperprolactinaemia
↓ testosterone
↓ LH, normal FSH
↓ cortisol & ACTH
Normal TSH
Constitutional, polyarthritis, peripheral neuropathy, pulmonary PR3 +ve
46 50 F Pituitary enlargement DI
Hyperprolactinaemia
Hypogonadism
Polyarthritis, ocular, mucocutaneous, ENT ANCA +ve
Duzgun 2005 [45] 15864593 1 47* 47 F Loss of PS DI
Anterior pituitary hormones normal
Polyarthritis, pulmonary, ENT, renal PR3 +ve
Seror 2006 [16] 16523054 3 45 50 F Nodular pituitary enlargement. Homogenous contrast-enhancement. Loss of PS DI
Hyperprolactinaemia
Thyrotropic deficiency
Hypogonadism
Corticotropic deficiency
ENT, ocular, mucocutaneous PR3 +ve
26 41 F Nodular pituitary enlargement. Contrast- enhancement DI
Normal anterior pituitary hormones
ENT, arthralgia, ocular, renal PR3 +ve
55 57 M Pituitary enlargement & central necrosis. Heterogenous enhancement. DI
Hyperprolactinaemia
Panhypopituitarism
Peripheral neuropathy, pulmonary, retinal vasculitis, digital, cerebral and renal infarcts PR3 +ve
Spisek 2006 [46] 16322901 1 30* 29 M Sellar cystic lesion DI
↓ TSH
↓ LH & FSH
↓ ACTH
↓ IGF-1
ENT PR3 +ve
McIntyre 2007 [47] 17318440 1 22* 22 F Heterogeneous enhancing pituitary mass DI
Hypogonadism
Cutaneous, ocular, renal PR3 +ve
Thiryayi
2007 [48]
17188492 1 21* 21 F Sellar mass with central hypo-intensity DI (post-surgery)
Hypogonadism (post-surgery)
Constitutional, arthralgia cANCA +ve
Yong 2008 [49] 17492510 1 33* 33 M Pituitary stalk thickening. Contrast-enhancing nodule at the superior aspect
of the stalk.
Loss of PS.
DI
Hypogonadism
↓ ACTH
Normal prolactin
Normal TSH
Normal IGF-1
ENT PR3 +ve
Cunnington 2009 [50] 20107566 3 19 24 M Pituitary enlargement DI
Anterior pituitary hormones normal
Constitutional, ENT, pulmonary, ocular, cutaneous PR3 +ve
33 34 F Diffusely enlarged gland containing a poorly enhancing lesion with supra-sellar extension. Loss of PS. DI
Normal prolactin
Normal TSH
Constitutional, ENT cANCA+ve
26 35 M Diffusely enlarged pituitary and thickened stalk. DI
↓ TSH
Constitutional, ENT, pulmonary cANCA +ve
Xue 2009 [51] 19172275 1 63* 63 F Normal DI
TSH normal
LH & FSH normal
Constitutional, pulmonary, peripheral neuropathy PR3 +ve
Barlas 2011 [52] 21116602 1 35 37 F Anterior enlargement.
Central area with low signal on T1 and high signal on T2. Post contrast enhancement of pituitary and stalk. Loss of PS.
DI
Hyperprolactinaemia
ENT, pulmonary, polyarthritis cANCA +ve
Santoro 2011 [53] 22147097 1 53* 53 F Hypointensity of adenohypophysis on T1.
Hyperintense sectors on R side on T2.
Peripheral contrast-enhancement.
Stalk-thickening.
Loss of PS.
DI
↓ TSH
Hypogonadism
Normal prolactin
Polyarthritis, cutaneous, pulmonary, renal cANCA +ve
Tenorio- Jimenez 2011 [54] 22673710 1 23 38 F MRI: marked infundibular thickening, sellar mass with hypointensity on T1. Loss of PS Hyperprolactinaemia
↓ TSH
Hypogonadism
Post pituitary hormones unaffected
ENT, pulmonary, renal cANCA +ve (−ve by the time of sellar manifestations)
Hughes 2013 [55] 23186961 1 N/A 30 F Sellar mass Panhypopituitarism Ocular N/A
Pereira 2013 [56] 22898089 1 48* 48 F Appearances of pituitary microadenoma, but histopathlogy revealed
necrotizing
granulomatous
inflammation
Hyperprolactinaemia
↓ TSH
Post pituitary hormones unaffected
ENT N/A
Kapoor 2014 [20] 25077899 8 N/A 67 F Peripherally enhancing cystic sellar mass compressing the stalk Hyperprolactinaemia
Hypogonadism
Normal TSH
ENT, renal 7/8 cases PR3 +ve
N/A 48 F Multiple non-enhancing cystic areas in the pituitary, convexity of superior margin of pituitary DI
↓ prolactin
↓ TSH
Hypogonadism
Corticotropic deficiency
ENT, pulmonary, cutaneous
N/A 28 F Sellar mass with large zone of central non-enhancement and peripheral enhancement. Stalk displacement DI
Hypogonadism
Normal prolactin
Normal TSH
Normal IGF-1
Normal cortisol
ENT, pulmonary, renal
N/A 55 M Sellar mass with suprasellar extension DI
Hypogonadism
Normal prolactin
Normal TSH
Normal cortisol
Normal IGF-1
ENT, pulmonary, renal, cutaneous, joints
N/A 35 M Necrotic sellar mass with peripheral enhancement & suprasellar extension. Thickened contrast-enhancing stalk DI
↓ TSH
Hypogonadism
Normal prolactin
Normal cortisol
Normal IGF-1
ENT
N/A 54 M Enlarged pituitary measuring 12 mm, with heterogeneous enhancement. Slight diffuse thickening of the stalk DI
↓ TSH
Hypogonadism
Normal prolactin
Normal IGF-1
ENT, pulmonary, renal, cardiac
N/A 68 M Homogeneously enhancing sellar mass, extending into the cavernous sinus bilaterally. Stalk preserved. ↓ prolactin
↓ TSH
Hypogonadism
↓ IGF-1
ENT, joints
N/A 28 F Sellar mass extending into the suprasellar cistern, with low T2 signal in the periphery and a bright centre. Peripheral enhancement with central cystic change. Thickening of pituitary stalk DI
Normal prolactin
Normal TSH
Normal cortisol
Normal IGF-1
ENT
De Parisot 2015 [19] 25906106 9 46* 46 F Enlarged posterior pituitary. Infiltration of posterior pituitary. Loss of PS DI ENT, ocular  
60 70 M Normal ↓ TSH
Hypogonadism
↓ IGF-1
Normal prolactin
ENT, peripheral neuropathy  
23 24 F Enlarged pituitary. Irregularity of infundibulum. Heterogeneous enhancement of anterior pituitary. Loss of PS DI
↓ TSH
Hypogonadism
Normal prolactin
Normal IGF-1
ENT  
24* 24 M Enlarged infundibulum DI
Hyperprolactinaemia
↓ TSH
Hypogonadism
Normal IGF-1
Renal, ocular, joints, gastro-intestinal  
66 77 M Enlarged pituitary. Stalk infiltration.
Loss of PS.
↓ TSH
Hypogonadism
↓ IGF-1
Normal prolactin
None  
67 68 F Normal DI
Hyperprolactinaemia
Normal TSH
Normal LH & FSH
Normal IGF-1
ENT  
28 42 F Heterogeneous enhancement of pituitary DI
Hyperprolactinaemia
Hypogonadism
↓ ACTH
ENT, lung  
55 57 M Sellar mass, heterogeneous.
Enhancement. Enlargement and infiltration of stalk. Loss of PS
DI
Hyperprolactinaemia
↓ TSH
Hypogonadism
↓ ACTH
Pulmonary  
46 50 F Enlargement and infiltration of pituitary. Heterogeneous enhancement, contact with optic chiasm DI
Hyperprolactinaemia
Hypogonadism
Normal TSH
Normal IGF-1
ENT, ocular  
Eli 2016 [22] 27521731 1 32* 29 F Homogenously enhancing sellar mass. Thickened stalk. Hyperprolactinaemia ENT, pulmonary MPO +ve
Esposito 2017 [57] 28540625 3 37* 37 F Sellar mass extending into the suprasellar cistern with peripheral enhancement and central cystic lesion. Stalk deviation. DI
Hypogonadism
GH deficiency
Constitutional, ENT PR3 +ve
36* 36 F Cystic pituitary mass DI
Anterior pituitary function normal
Constitutional, myalgia, ENT, pulmonary PR3 +ve
32* 32 F Sellar mass with homogeneous. Thickening of the pituitary stalk DI
Anterior pituitary function normal
ENT PR3 +ve
  1. Abbreviations: ACTH adrenocorticotropic hormone, cANCA cytoplasmic pattern ANCA staining, DI diabetes insipidus, GH growth hormone, IGF-1 insulin-like growth factor 1, LH luteinising hormone, FSH follicular stimulating hormone, MPO myeloperoxidase, N/A data not available, PMID PubMed ID, PR3 proteinase-3, PS posterior signal.
  2. *indicates cases where pituitary involvement was diagnosed prior to or at the time of GPA diagnosis
  3. For the purposes of the Table, a TSH within the reference range but inappropriately low for the T4 has been included in the category “↓ TSH”