Research Article

Pituitary Stalk Germ Cell Tumors: Retrospective Case Series and Literature Review

Table 3

Cases of intracranial germ cell tumors with pituitary stalk involvement in the published literature.

StudyPatientManifestation (duration)Neuroendocrine disturbanceInitial MRIInitial tumor markersInitial diagnosis (method)Initial therapeutic strategy (observation duration)Aggravated manifestationsRepeat MRIRepeat markersDiagnosis (method)Neuroendocrine disturbance

Mooth 1997 [13]5.8FPU/PD (5 months)CDI, normalNormalNormalNA(8 months)NAPST and pineal massNormalGE (pathology)NA
10.5FPU/PD and SS (4 years)CDI and GHDNormalNormalNA(3 months)NAPST and sellar massElevated CSF hCGGE (pathology)NA
10.5MPU/PD and SS (2 months)CDI, GHD, and HPRLPSTNormalNA(9 months)NAPST and suprasellar massElevated CSF hCGGE (pathology)NA
11.8FPU/PD and SS (2 months)CDI and HPAPSTNormalGranuloma (pathology)(11 months)NAExtension of the lesionNormalGE (pathology)NA
12.9FPU/PD (25 months)CDI, HPG, HPT, HPA, and HPRLPSTNormalNA(14 months)NAProgressive PSTNormalGE (pathology)NA
18.1FPU/PD and DP (17 months)CDI, HPA, HPG, GHD, and HPRLPSTNormalNA(8 months)NANAElevated CSF hCGGE (pathology)NA

Bettendorf 1999 [10]8FPU/PD, SS, and loss of appetite (2 years)CDI, HPG, GHD, HPT, and HPRLPST and optic chiasma involvementNormalLYH (pathology)Dexamethasone(18mg/d) for 5 months (5 months)VI, headache, and vomitingMultiple intracranial lesionsNAGE (pathology)CDI, HPA, HPT, and GHD
Fehn 1999 [11]12FPU/PD and SS (2 years)CDI, HPG, GHD, HPT, and HPRLPST and enlarged pituitaryNormalLYH (pathology)Dexamethasone for 5 months (1 year)VI, headache, and vomitingMultiple intracranial lesionsNAGE (pathology)CDI and panhypopituitarism
Leger 1999 [14]13PU/PD and SS (3.8 years)CDIPST, enlarged pituitary, and sellar massNA-(1.7 years)NAPST and enlarged pituitaryNAGE (pathology)GHD, HPA, HPT, and HPG
7PU/PD and SSCDIPSTNANA(1.1 years)NAPST and enlarged pituitaryNAGE (pathology)GHD, HPA, and HPT
13PU/PD and SS (2 years)CDIPST and enlarged pituitaryNANA(6 months)NAPST and enlarged pituitaryNAGE (pathology)GHD, HPA, HPT, and HPG
8PU/PD and SSCDIPST and enlarged pituitaryNANA(1.8 years)NAPST and enlarged pituitaryNAGE (pathology)GHD

Endo 2002 [15]12MPU/PD, fatigue, and loss of appetiteCDI and panhypopituitarismSuprasellar and intrasellar mass with optic chiasma involvement and cavernous sinus invasionNormalGranuloma (pathology)Glucocorticoid for 6 monthsPU/PD and progressing VIRelapse and extensionNAGE (pathology)Hormone replacement without details
Prosch 2006 [16]9.5FPU/PD, vomiting, and fatigue (1year)CDI and GHDPST and absent posterior pituitaryNormalLCH (clinical)GHRT for 7 months, prednisone (40mg/m2), and vinblastine for 11 months (24 months)NAPST and enlarged pituitaryNAGE (pathology)CDI and panhypopituitarism
Ozbey 2006 [18]24FPolymenorrhea and headacheHPT, HPRL, and HPGPST and suprasellar and intrasellar massElevated serum hCGLYH (clinical)Glucocorticoid for 3 weeks (3 months)NANo regressionElevated serum hCGGE (pathology)Panhypopituitarism
Mikami-Terao 2006 [17]13FPU/PD, headache, SS, fatigue, and DP (2 years)CDI, HPG, HPT, and GHDPST and absent posterior pituitaryNormalLYH (pathology)Prednisolone (1mg-0.32/kg/d) for 3 months (12 months)VIEnlarged mass in the pituitary stalk and hypothalamusNormalGE (pathology)CDI, HPA, HPT, HPG, and GHD
Edouard 2009 [19]10FPU/PD, headache, and DP (1year)CDI, HPT, and GHDPST and absent posterior pituitaryNormalLYH (pathology)GHRT for 5 months (9 months)NAEnlarged mass in the pituitary stalk and hypothalamusNAGE (pathology)CDI and HPT
4.5MPU/PD and SS (1 year)CDI and GHDIsolated PSTNormalLYH (pathology)GHRT for 3 years (6 years)CDI, GHD, HPA, and HPTProgressive PSTNormalGE (pathology)CDI, HPA, HPT, and GHD
Jevalikar 2012 [21]10MPU/PD (6 months)CDI and GHDAbsent signal of posterior pituitaryNormalLYH (pathology)GHRT for 2 months (16 months)Headache and Parinaud's signEnlarged infundibular, pineal massElevated CSF hCGGE (pathology)CDI, HPA, HPT, and GHD
Guzzo 2012 [23]24FPU/PD, amenorrhoea, and fatigue (1 year)CDI, HPA, HPT, HPG, and HPRLPST and suprasellar massNormalLYH (pathology)NANANANAGE (pathology)NA
Terasaka 2012 [20]40FPU/PD, amenorrhoea, polygalactia, headache, and VI (3 years)CDI, HPA, HPT, and HPGPST and intrasellar massNormalLYH (pathology)Hydrocortisone (1000mg with gradient decrease) for 2 weeksDeterioratedEnlarged massNAGE (pathology)HormoNAl replacement without details
Robison 2013 [12]5PU/PDCDIIsolated PSTNormalLYH (pathology)NA (6 years)NANAElevated CSF hCGGE (pathology)APD
19PU/PDCDI and hypopituitarismIsolated PSTElevated CSF hCGGE (pathology)NA
10PU/PDCDIIsolated PSTNormalGE (pathology)APD
11PU/PDCDIIsolated PSTNormalGE (pathology)APD

Zilbermint 2014 [22]13FPU/PD, fatigue, and amenorrhoea (1 year)CDI, HPA, HPT, HPG, and HPRLPST and extending to hypothalamusNormalGE (pathology)CDI, HPA, and HPT
Graaf 2020 [9]12FPU/PD (3 years)CDI, HPA, HPT, and HPGPST, sellar-suprasellar mass, and absent posterior pituitaryElevated CSF hCGGranuloma (pathology)Glucocorticoid (1mg/kg/d) for 3 months (36 months)CDI, VI, headache, and vomitingSellar mass and enhancement of the ependyma and pineal glandElevated CSF hCGGE (pathology)CDI, HPA, and HPG
Dias 2020 [24]27MPU/PD (1 year)CDIPST, absent posterior pituitary, and pineal cystElevated CSF hCGNeurohypophysitis (clinical)Methylprednisolone (500mg/week to 250mg/week) for 3 months (1 month)AnejaculationEnlarged PST, absent posterior pituitary, and pineal cystElevated CSF hCGGE (pathology)CDI, HPA, HPT, HPG, GHD

Patient: M, male; F, female; manifestations: PU/PD, polydipsia and polyuria; SS, short stature, DP, delayed puberty; HH, hypogonadotropic hypogonadism; VI, visual impairment; neuroendocrine disturbance: CDI, central diabetes insipidus; HPA, hypothalamic pituitary adrenal axis; HPT, hypothalamic pituitary thyroid axis; HPG, hypothalamic pituitary gonadal axis; HPRL, hyperprolactinemia, GHD, growth hormone deficiency; APD, anterior pituitary hormone deficiency; MRI: PST, pituitary stalk thickening; tumor markers: hCG, human chorionic gonadotropin; AFP, alpha-fetoprotein; CSF, cerebrospinal fluid; diagnosis: LYH, lymphocytic hypophysitis; LCH, Langerhans cell histocytosis; GE, germinomas; NGGCT, nongeminomatous germ cell tumors; therapy: GHRT, growth hormone replacement treatment; NA, not applicable.