DOI:10.2214/AJR.05.2027
AJR 2007; 188:W223-W232
© American Roentgen Ray Society
Anatomic and Pathologic Spectrum of Pituitary Infundibulum Lesions
Bronwyn E. Hamilton1,2,
Karen L. Salzman1 and
Anne G. Osborn1
1 Department of Radiology, University of Utah, Salt Lake City, UT.
2 Present address: Department of Radiology, Oregon Health & Science
University, 3181 SW Sam Jackson Park Rd., Portland, OR 97239.
Received November 22, 2005;
accepted after revision March 15, 2006.
Address correspondence to B. E. Hamilton
(hamiltob{at}ohsu.edu).
B. E. Hamilton is a consultant for and receives loyalties from Amirsys.
CME This article is available for CME credit. See
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Abstract
OBJECTIVE. The pathologic spectrum of pituitary infundibulum disease
is diverse. We reviewed 65 infundibular lesions in 44 adult and 21 pediatric
patients and summarized their imaging features and clinical presentation.
CONCLUSION. The spectrum of pathology involving the pituitary
infundibulum is broad yet distinct from other pathology in the sella and
parasellar region. Pituitary stalk lesions can be grouped into three
categories: congenital and developmental, inflammatory and infectious, and
neoplastic. Knowledge of the imaging appearance of diseases specific to adults
and to children is important for accurate diagnosis and treatment.
Keywords: anatomy central nervous system neuroradiology pituitary lesions
Introduction
The spectrum of pathology involving the pituitary infundibulum is
broad yet distinct from other pathology in the sella and parasellar region.
Infundibular pathology is usually considered with more general sellar and
parasellar disease.
We reviewed 65 infundibular lesions and summarized their imaging features
and clinical presentation. Lesions affecting the pituitary infundibulum were
grouped into three broad categories: congenital and developmental,
inflammatory and infectious, and neoplastic.
Materials and Methods
A retrospective review of our institutional case archive from 1995 to 2003
revealed 65 infundibular lesions. Imaging and clinical presenting features
were evaluated to better characterize the pathologic spectrum of pituitary
infundibulum disease. For the purposes of this study, patients older than 21
years were considered to be adults. Patients 21 years or younger were
considered pediatric.
High-resolution MRI was performed in 45 patients using 3-mm sections
through the pituitary gland with unenhanced T1-weighted and contrast-enhanced
T1-weighted sagittal and coronal images, and coronal 3-mm T2-weighted images
through the gland. Three patients underwent dynamic gadolinium-enhanced MRI
through the gland. Routine enhanced brain MRI using 5-mm axial slices was
performed in 15 patients. Contrast-enhanced CT of the brain was performed in
five patients.
Imaging studies were reviewed for morphology, signal intensity, and
enhancement characteristics of the pituitary gland and stalk. Abnormalities
relevant to the primary disease process were noted when present. These
included midline malformations in pituitary dwarfism (absent septum
pellucidum, hypoplastic optic and olfactory nerves) and secondary mass lesions
(periventricular, pineal masses) in neoplasms. Clinical presenting features
were noted in all cases. Diagnosis was established by histopathology in 26
patients and by characteristic imaging findings or associated clinical
features in 39 patients.
Results
Infundibular pathology included a broad range of neoplasms, inflammatory
and infectious disorders, and congenital and developmental lesions
(Fig. 1). Neoplasms slightly
predominated over other disease categories, accounting for 24 (37%) of 65
cases overall.
Pediatric infundibular lesions accounted for 21 (32%) of 65 lesions. We
found 13 congenital lesions (all pituitary hypoplasias) and eight tumors in
children. Pediatric neoplasms included four cases of Langerhans cell
histiocytosis (LCH), two germinomas, and one case each of metastatic
glioblastoma multiforme and primitive neuroectodermal tumor. The pediatric
pathologic spectrum is presented in Figure
2.
Adult infundibular pathology accounted for 44 (68%) of all lesions. These
included 19 inflammatory lesions, 16 neoplasms, and nine congenital or
developmental lesions (Fig. 3).
Infundibuloneurohypophysitis (INH) was the most common inflammatory lesion in
adults, accounting for 12 of 44 cases. Other inflammatory lesions included
sarcoidosis (n =6) and one intracranial Whipple's disease confined to
the pituitary stalk and the median eminence of the hypothalamus.
Neoplasms were found in 16 (36%) of 44 adults. Five cases of metastatic
disease were identified. Primary cancers were lung cancer in three patients,
breast cancer in one, and unknown in one. Three pituitary adenomas, one
primary and two recurrent, involved the infundibulum. Only adenomas centered
in the stalk and not the pituitary gland proper were included in this series.
Two cases each of germinoma, lymphoma, and leukemia were found. Leukemias
consisted of one acute myelogenous leukemia and one chronic myelogenous
leukemia. Two primary pituicytomas were also found.
Congenital and developmental lesions accounted for nine (20%) of 44 adult
cases. Five pituitary dwarfs, two cysts, one hypophyseal duplication, and one
vascular lesion were identified.
Discussion
The goal of this study is to review the diverse spectrum of pituitary
infundibular disease. The retrospective nature of data collection and our own
institutional referral bias were limitations of this study that did not permit
an accurate assessment of specific disease prevalence. Despite this, some
conclusions may still be drawn regarding specific pathologic entities and
corresponding therapeutic implications. Inflammatory disease in adults, for
example, is likely underappreciated as a cause of stalk thickening, given its
surprisingly high frequency in this series.
Normal Anatomy
The pituitary infundibulum is an anatomically complex area
(Fig. 4). We briefly review
normal development and histology of the pituitary gland and stalk and discuss
the normal imaging appearance of the stalk.

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Fig. 4 Sagittal graphic of pituitary stalk anatomy: infundibulum is
composed of infundibular stem (curved arrow), median eminence of
hypothalamus, from which it arises, and pars tuberalis (straight
arrow). (Reprinted with permission from Harnsberger H. Head and neck
digital teaching file. Salt Lake City, UT: Amirsys, 2002
[16])
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Normal Pituitary Development
The pituitary gland forms after primary neurulation and has two distinct
embryologic origins. The anterior gland (adenohypophysis) arises from
invaginating placoid ectoderm in the roof of the stomodeum at approximately 3
weeks of gestation. This invagination is also known as Rathke's pouch, which
normally loses its connection to the oral cavity in the second gestational
month [1].
Both the posterior pituitary (neurohypophysis) and the median eminence of
the hypothalamus arise from neuroectoderm in the floor of the forebrain. The
infundibular stalk is a variably hollow tube that arises from the ventromedial
hypothalamus and is contiguous with the infundibular recess of the third
ventricle. The pars tuberalis encircles the infundibular stem as it enters the
adenohypophysis. The pars intermedia separates the adenohypophysis from the
posteriorly located neurohypophysis
[1].
Normal Histology
The histopathologic composition of the neurohypophysis is complex. Two
specialized modified glial cells, the tanycyte and the pituicyte, appear to
play a supportive role for the axons of vasopressin and oxytocin, producing
neurons, the cell bodies of which arise in the hypothalamus
[2]. Both of these cells appear
to be responsible for specific tumors in the suprasellar hypophyseal region:
pituicytomas and the recently described tanycytoma
[3].
Normal Imaging
The normal pituitary stalk tapers from superior to inferior. It measures
3.25 ± 0.56 (SD) mm at the level of the optic chiasm and narrows to
1.91 ± 0.40 mm at its insertion into the pituitary gland
[4]. The signal intensity of
normal stalk on T1 weighting is usually less than that of the optic chiasm and
is less than that of normal neurohypophysis in all cases. The stalk lacks a
blood-brain barrier and thus enhances intensely. Deviation or tilt of the
infundibulum is common and does not necessarily imply underlying disease.
The normal hyperintensity of the posterior pituitary gland on T1-weighted
sequences is believed to be related to vasopressin neurosecretory granules or
lipoid bodies (lysosomes) in pituicytes that are normally located in the
neurohypophysis [5].
Developmental and Congenital Lesions
Pituitary hypoplasia was the single most common infundibular abnormality we
found in children (13/21). Five adults were also imaged with pituitary
hypoplasia. Patients present clinically with dwarfism or growth hormone
deficiency. All showed abnormal-appearing stalks with posterior pituitary
ectopia, seen as a bright spot at the median eminence on unenhanced
T1-weighted images. Twelve of these patients had hypoplasia or absence of the
stalk. In two cases, the stalk was so attenuated that it was identified only
after contrast administration. Six patients had short, thickened stalks. One
patient with an absent stalk presented clinically with anosmia and was noted
to have absence of the septum pellucidum, although vision and the optic nerves
were normal (Fig. 5A,
5B).

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Fig. 5A 17-year-old boy with growth hormone deficiency and anosmia.
(Reprinted with permission from Harnsberger H. Head and neck digital
teaching file. Salt Lake City, UT: Amirsys, 2002
[16]) Sagittal (A) and
coronal (B) unenhanced T1-weighted images through sella reveal
pituitary hypoplasia. Pituitary gland and sella turcica are small. Note
ectopic neurohypophyseal T1 hyperintensity (open arrow). Infundibular
stem is always abnormal; in this case, it is markedly attenuated (solid
arrow). Stalk may be well seen only after gadolinium administration.
Associated abnormalities are frequent, as in this patient in whom septum
pellucidum is absent.
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Fig. 5B 17-year-old boy with growth hormone deficiency and anosmia.
(Reprinted with permission from Harnsberger H. Head and neck digital
teaching file. Salt Lake City, UT: Amirsys, 2002
[16]) Sagittal (A) and
coronal (B) unenhanced T1-weighted images through sella reveal
pituitary hypoplasia. Pituitary gland and sella turcica are small. Note
ectopic neurohypophyseal T1 hyperintensity (open arrow). Infundibular
stem is always abnormal; in this case, it is markedly attenuated (solid
arrow). Stalk may be well seen only after gadolinium administration.
Associated abnormalities are frequent, as in this patient in whom septum
pellucidum is absent.
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One hypophyseal duplication was discovered in an asymptomatic adult
(Fig. 6). Hypophyseal
duplication results in complete duplication of the entire gland, including the
infundibulum. Most reported cases in the literature are associated with
midline facial or oral anomalies, particularly the median cleft facial
syndrome, and many die in infancy. Hypertelorism is invariably present, and
hypothalamic thickening may be seen
[6].

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Fig. 6 Sagittal T1-weighted contrast-enhanced image through sella is
diagnostic of hypophyseal duplication in this 69-year-old asymptomatic man.
Complete pituitary gland and stalk (arrows) are duplicated.
(Reprinted with permission from Harnsberger H. Head and neck digital
teaching file. Salt Lake City, UT: Amirsys, 2002
[16])
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Two unenhancing cystic masses confined to the infundibular stalk were
thought to represent Rathke cleft or pars intermedia cysts. One case of
infundibular thickening in an asymptomatic adult appeared to be secondary to a
vascular malformation resulting in venous congestion.
Inflammatory Lesions: INH
Inflammatory and infectious infundibular lesions were found exclusively in
adults. INH was the most common inflammatory lesion, accounting for 12 cases
in this series (Fig. 7A,
7B). Imaging features of INH
include an enhancing masslike thickening of the pituitary infundibulum. The
normal T1 hyperintensity of the neurohypophysis is almost invariably absent
[7].

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Fig. 7A Adult male with infundibuloneurohypophysitis (INH). (Reprinted with
permission from Harnsberger H. Head and neck digital teaching file.
Salt Lake City, UT: Amirsys, 2002
[16]) Coronal (A) and
sagittal (B) contrast-enhanced T1-weighted images through sella reveal
typical imaging findings of INH. Note masslike thickening of infundibulum
(arrow).
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Fig. 7B Adult male with infundibuloneurohypophysitis (INH). (Reprinted with
permission from Harnsberger H. Head and neck digital teaching file.
Salt Lake City, UT: Amirsys, 2002
[16]) Coronal (A) and
sagittal (B) contrast-enhanced T1-weighted images through sella reveal
typical imaging findings of INH. Note masslike thickening of infundibulum
(arrow).
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The earliest reported cases of hypophysitis (or adenohypophysitis) involved
the anterior gland and were typically seen in women in late pregnancy or
shortly postpartum (Fig. 8A,
8B). Later, it became clear
that a pathologically similar process involved the stalk (INH or
neurohypophysitis); this process is also more common in women
[8]. Although typically seen in
young adults, INH and adenohypophysitis have occurred in children and elderly
patients. Pathologic features of INH and hypophysitis include chronic
inflammatory changes, hyaline fibrosis, and infiltration of the pituitary
gland and stalk by plasma cells or lymphocytes.

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Fig. 8A 23-year-old postpartum woman with hypophysitis and atypical
infundibuloneurohypophysitis involving pituitary gland and stalk. (Reprinted
with permission from Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002
[16]) Coronal (A) and
sagittal (B) contrast-enhanced T1-weighted images through sella. Note
masslike enlargement of entire pituitary gland and stalk. Neurohypophyseal
involvement was supported by clinical presentation of diabetes insipidus. With
this degree of involvement, imaging appearance is indistinguishable from
macroadenoma; however, histopathologic evaluation confirmed diagnosis of
hypophysitis.
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Fig. 8B 23-year-old postpartum woman with hypophysitis and atypical
infundibuloneurohypophysitis involving pituitary gland and stalk. (Reprinted
with permission from Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002
[16]) Coronal (A) and
sagittal (B) contrast-enhanced T1-weighted images through sella. Note
masslike enlargement of entire pituitary gland and stalk. Neurohypophyseal
involvement was supported by clinical presentation of diabetes insipidus. With
this degree of involvement, imaging appearance is indistinguishable from
macroadenoma; however, histopathologic evaluation confirmed diagnosis of
hypophysitis.
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INH is increasingly recognized radiographically and clinically. It may be
the underlying pathology in many cases that have historically been categorized
as idiopathic central diabetes insipidus; it is believed to be
autoimmune-mediated. Central diabetes insipidus is characterized by deficient
arginine-vasopressin (AVP) synthesis from the hypothalamus or deficient
secretion from the neurohypophysis.
Autoimmune central diabetes insipidus is diagnosed by documenting the
presence of serum autoantibodies to AVP-secreting cells or by the coexistence
of autoimmune polyendocrine syndromes. Clinical information is helpful in
determining the cause of stalk thickening in adults with central diabetes
insipidus. Age of onset of less than 30 years suggests INH as the likely cause
of diabetes in about 80% of cases. Patients who also have a history of
antecedent autoimmune disease, such as Hashimoto's thyroiditis, atrophic
gastritis, or pernicious anemia, raise the likelihood of INH as the underlying
cause to 99% [9].
Desmopressin or steroid treatment can result in clinical and imaging
improvement or even disease regression in some cases of INH or hypophysitis.
This is particularly true in patients with partial central diabetes insipidus,
who have less stalk or glandular destruction
[9]. Surgery may therefore be
best reserved for patients with persisting local mass effect that is
refractory to medical management.
This paradigm is not useful in very young children in whom LCH is a
frequent cause of stalk thickening and central diabetes insipidus. Patients
with LCH are generally younger than 30 years and have high serum AVP-secreting
cells. Some authors think antibodies in patients with LCH may be a secondary
autoimmune process [9].
Regardless, INH or hypophysitis is usually a disease of young adults.
Infectious Lesions: Granulomatous and Infectious Causes
Sarcoidosis involved the stalk in six cases in this series, all of whom
were adults. Sarcoidosis rarely presents with isolated clinical
hypothalamic-pituitary involvement, although stalk thickening on imaging is
not rare in patients with CNS involvement (Fig.
9A,
9B).

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Fig. 9A 36-year-old man with sarcoidosis. (Reprinted with permission from
Harnsberger H. Head and neck digital teaching file. Salt Lake City,
UT: Amirsys, 2002 [16]) Axial
(A) and coronal (B) contrast-enhanced T1-weighted images through
pituitary stalk (arrow).
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Fig. 9B 36-year-old man with sarcoidosis. (Reprinted with permission from
Harnsberger H. Head and neck digital teaching file. Salt Lake City,
UT: Amirsys, 2002 [16]) Axial
(A) and coronal (B) contrast-enhanced T1-weighted images through
pituitary stalk (arrow).
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One adult case of stalk thickening in this series was diagnosed at
pathology as Whipple's disease (Fig.
10A,
10B). Although rare, this
finding has been previously reported
[10]. Other granulomatous
processes that may involve the pituitary stalk include tuberculosis and
Wegener's disease.

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Fig. 10A 42-year-old man with Whipple's disease. (Reprinted with permission
from Harnsberger H. Head and neck digital teaching file. Salt Lake
City, UT: Amirsys, 2002 [16])
Axial (A) and sagittal (B) contrast-enhanced T1-weighted images
show isolated thickening and enhancement of median eminence and pituitary
infundibulum (arrow), subsequently proven at histopathology to be
Whipple's disease.
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Fig. 10B 42-year-old man with Whipple's disease. (Reprinted with permission
from Harnsberger H. Head and neck digital teaching file. Salt Lake
City, UT: Amirsys, 2002 [16])
Axial (A) and sagittal (B) contrast-enhanced T1-weighted images
show isolated thickening and enhancement of median eminence and pituitary
infundibulum (arrow), subsequently proven at histopathology to be
Whipple's disease.
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Neoplasms in Children
Neoplasms accounted for eight (38%) of 21 pediatric infundibular
abnormalities (Fig. 2). The
most common pediatric infundibular tumor is LCH. Controversy exists regarding
the most appropriate categorization of LCH, which is variably classified as a
neoplasm and as an inflammatory (granulomatous) process. Meningeal involvement
and choroid plexus lesions, although not seen in this series, are secondary
abnormalities that provide a clue to diagnosis when present
[11]. Central diabetes
insipidus is a common indication for imaging the pituitary hypothalamic axis.
Prior reports suggest a mass is found in two thirds of patients with central
diabetes insipidus, with LCH the most common diagnosis in children
[5].
In nearly all cases of LCH, the normal T1 hyperintensity of the
neurohypophysis is absent
[11]. This nonspecific finding
was present in all cases in this series. The most common specific finding is
stalk thickening (Fig. 11A,
11B). Erdheim-Chester disease
is a rare condition that is also characterized by histiocytic infiltration of
the pituitary stalk [5].

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Fig. 11A 14-year-old boy with Langerhans cell histiocytosis (LCH). (Reprinted
with permission from Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002
[16]) Sagittal (A) and
coronal (B) contrast-enhanced T1-weighted images reveal focal
infundibular thickening in LCH, which was most common pediatric cause of
infundibular masslike thickening in this series (arrow). Posterior
pituitary ectopia was seen in all patients.
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Fig. 11B 14-year-old boy with Langerhans cell histiocytosis (LCH). (Reprinted
with permission from Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002
[16]) Sagittal (A) and
coronal (B) contrast-enhanced T1-weighted images reveal focal
infundibular thickening in LCH, which was most common pediatric cause of
infundibular masslike thickening in this series (arrow). Posterior
pituitary ectopia was seen in all patients.
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Four germinomasin two pediatric patients and two young
adultsall showed stalk thickening and an absent posterior hypophyseal
bright spot. CSF seeding and secondary pineal region masses may be important
clues to diagnosis (Fig. 12).
Metastatic glioblastoma multiforme and primitive neuroectodermal tumor were
isolated causes of stalk masses in two cases.

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Fig. 12 Sagittal contrast-enhanced T1-weighted image in 40-year-old woman
reveals masslike thickening of pituitary infundibulum in intracranial
germinoma (straight arrow). Recognition of subtle secondary pineal
mass (curved arrow) is highly suggestive of diagnosis. (Reprinted
with permission from Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002
[16])
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Neoplasms in Adults: Secondary Tumors
Neoplastic conditions of the stalk in the adult population are diverse
(Fig. 3). Metastatic disease
was the most common adult infundibular tumor in this series. Metastatic
disease to the pituitary is frequent in autopsy series, with breast and lung
the most common primary sites. Metastatic disease usually presents in older
patients, with a tendency for local invasion and rapid growth
[12].
Leukemia (one acute myelogenous leukemia, one chronic myelogenous leukemia)
and lymphoma each occurred in two adults. These causes of stalk thickening are
not usually a diagnostic dilemma because the diagnosis is typically known long
before the development of this late complication. Leukemia patients with stalk
involvement present clinically with diabetes insipidus, and the prognosis is
poor. Lymphoma may present intracranially as isolated stalk thickening or in
conjunction with periventricular enhancing masses
(Fig. 13). Pituitary adenomas
occasionally arise in the infundibulum, as in three patients in this series
(Fig. 14A,
14B).

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Fig. 13 Axial contrast-enhanced T1-weighted image in 62-year-old man shows
lymphoma of pituitary infundibulum (solid arrow). Although isolated
stalk involvement may be seen, enhancing periventricular masses in this
patient improved diagnostic specificity (open arrow). (Reprinted with
permission from Harnsberger H. Head and neck digital teaching file.
Salt Lake City, UT: Amirsys, 2002
[16])
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Fig. 14A 34-year-old woman with elevated prolactin. (Reprinted with
permission from Harnsberger H. Head and neck digital teaching file.
Salt Lake City, UT: Amirsys, 2002
[16]) Sagittal
contrast-enhanced T1-weighted image of infundibular adenoma shows that,
superiorly, mass (open arrow) is inseparable from pituitary
infundibulum (solid arrow).
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Fig. 14B 34-year-old woman with elevated prolactin. (Reprinted with
permission from Harnsberger H. Head and neck digital teaching file.
Salt Lake City, UT: Amirsys, 2002
[16]) Coronal
contrast-enhanced T1-weighted image shows prolactinoma (white arrow)
arising from stalk (black arrow).
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Neoplasms in Adults: Primary Glial Tumors
Primary tumors of the neurohypophysis are rare. Two primary gliomas of the
infundibulum were found in this series, both pituicytomas. One occurred in a
33-year-old woman presenting with delayed growth and panhypopituitarism (Fig.
15A,
15B). The other was found in a
35-year-old man with normal hormone levels. No other primary gliomas occurred
in our series. Hypothalamic-chiasmatic astrocytomas may occur primarily in the
stalk or involve the stalk by secondary extension. Ependymoma and pleomorphic
xanthoastrocytoma of the stalk have also been described. Although rare,
primary gliomas should be included in the differential diagnosis of stalk
masses.

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Fig. 15A 35-year-old man with pituicytoma. (Reprinted with permission from
Harnsberger H. Head and neck digital teaching file. Salt Lake City,
UT: Amirsys, 2002 [16])
Sagittal (A) and coronal (B) contrast-enhanced T1-weighted
images show that pituitary infundibulum (arrows) is closely
associated with mass.
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Fig. 15B 35-year-old man with pituicytoma. (Reprinted with permission from
Harnsberger H. Head and neck digital teaching file. Salt Lake City,
UT: Amirsys, 2002 [16])
Sagittal (A) and coronal (B) contrast-enhanced T1-weighted
images show that pituitary infundibulum (arrows) is closely
associated with mass.
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Neurohypophyseal-Specific Glial Tumors: Pituicytoma, Tanycytoma, and Granular Cell Tumor
A number of gliomas have been described as arising in the pituitary stalk;
however, two types in particular are highly location-specific to the stalk:
pituicytomas (sometimes called infundibulomas) and tanycytomas. Occasionally,
granular cell tumors (also called granular cell myoblastoma or choristoma)
have been seen in this location, although they are somewhat less specific to
the stalk. Confusion has arisen in the literature regarding these unusual
tumors because of overlapping terminology and pathologic mischaracterization
in the past [13].
Pituicytes are highly specialized glial cells found nearly exclusively in
the normal pituitary stalk and posterior gland. Pituicytomas have been
confused with several benign and malignant tumors. They may superficially
resemble pilocytic astrocytomas, meningiomas (particularly fibrous),
schwannomas, and adenomas on histopathology. These tumors may be difficult to
differentiate by imaging and histology. Improved morphologic characterization,
immunohistochemical techniques, and electron microscopy now aid in
differentiating these tumors pathologically
[13].
Pituicytomas most commonly occur in middle-aged men in the third to fifth
decade, although there is a documented case in an 83-year-old man
[14]. None have been described
in children. Common clinical presenting features of pituicytomas include
panhypopituitarism and fatigue.
Imaging features of pituicytomas include isodensity or isointensity to
normal brain and homogeneous contrast enhancement in most cases (Fig.
14A,
14B). These tumors are
localized to the pituitary stalk and posterior gland and frequently grow into
the suprasellar cistern, potentially compressing the optic nerves and
hypothalamus. The normal T1 hyperintensity of the neurohypophysis is typically
absent. Sellar expansion is uncommon and is a feature that may help
differentiate stalk tumors from adenomas.
Pituicytomas are benign tumors that have shown no recurrence after gross
total resection on long-term follow-up; thus, the correct diagnosis has
relevant implications for treatment and prognosis. Patients undergoing gross
total resection may be cured, requiring no adjuvant chemotherapy or radiation.
Recurrences in patients who have undergone incomplete resection have been
followed up conservatively
[13].
Tanycytomas are another recently described tumor in the
hypothalamic-suprasellar region, so named because of specific staining and
ultrastructural characteristics identical to normal tanycyte morphology. These
tumors may overlap pathologically with pilomyxoid astrocytomas, a recently
described tumor [3,
15] with histopathologic
features matching those of tanycytoma. Electron microscopy has not been
performed in these patients; thus, the most appropriate pathology terminology
remains unclear. Tanycytomas are characterized by large suprasellar masses;
their relationship to the pituitary stalk is difficult to determine.
Tanycytomas are clinically aggressive, with increased recurrence rates,
possibly accounting for the larger size at presentation than typically seen
with pituicytoma [3].
Conclusion
The spectrum of pathology involving the pituitary infundibulum is broad yet
distinct from other pathology in the sella and parasellar region. The
pituitary infundibulum presents a diagnostic imaging challenge because of its
small size and protean spectrum of disease processes.
Congenital and developmental infundibulum abnormalities usually have
characteristic findings on imaging. Primary glial tumors such as pituicytoma
and tanycytoma have distinctly different clinical courses; therefore,
including such tumors in the differential diagnosis of infundibular masses has
implications for correct histopathologic diagnosis and treatment.
Inflammatory disorders are likely underrecognized as a cause of masses of
the pituitary stalk in adults. Their inclusion in the differential diagnosis
of adult infundibular disease is important because it may allow the option for
noninvasive treatment and improved clinical course, particularly in patients
with INH or adenohypophysitis.
References
- Bannister L, section ed. Endocrine system. In: Bannister L, Berry
M, Williams P, eds. Gray's anatomy: the anatomical basis of
medicine and surgery, 38th ed. New York, NY: Churchill
Livingstone, 1995: 1883-1888
- Schultz A, Brat D, Oyesiku M, Hunter S. Intrasellar pituicytoma in
a patient with other endocrine neoplasms. Arch Pathol Lab
Med 2003; 125:527
-530
- Lieberman K, Chang J, Schelper R, et al. Tanycytomas: a newly
characterized hypothalamic-suprasellar and ventricular tumor. Am J
Neuroradiol 2003; 24:1999
-2004[Abstract/Free Full Text]
- Tien R, Newton T, McDermott M, Dillon W, Kucharczyk J. Thickened
pituitary stalk on MR images in patients with diabetes insipidus and
Langerhans cell histiocytosis. Am J Neuroradiol1990; 11:703
-708[Abstract]
- Tien R, Kucharczyk J, Kucharczyk W. MR imaging of the brain in
patients with diabetes insipidus. Am J Neuroradiol1991; 12:533
-542[Abstract]
- Ryals B, Brown D, Levin S. Duplication of the pituitary gland as
shown by MR. Am J Neuroradiol 1993;14
: 137-139[Abstract]
- Nishioka H, Ito H, Sano T, Ito Y. Two cases of lymphocytic
hypophysitis presenting with diabetes insipidus: a variant of lymphocytic
infundibulo-neurohypophysitis. Surg Neurol1996; 46:285
-290[CrossRef][Medline]
- Sato N, Sze G, Keigo E. Hypophysitis: endocrinologic and dynamic MR
findings. Am J Neuroradiol 1998;19
: 439-444[Abstract]
- Pivonello R, De Bellis A, Faggiano A, et al. Central diabetes
insipidus and autoimmunity: relationship between the occurrence of antibodies
to arginine vasopressin-secreting cells and clinical, immunological, and
radiological features in a large cohort of patients with central diabetes
insipidus of known and unknown etiology. J Clin Endocrinol
Metab 2003;88:1629
-1636[Abstract/Free Full Text]
- Brandle M, Ammann P, Spinas GA, et al. Relapsing Whipple's disease
presenting with hypopituitarism. Clin Endocrinol1999; 50:399
-403[Medline]
- Prayer D, Grois N, Prosch H, Gadner H, Barkovich AJ. MR imaging
presentation of intracranial disease associated with Langerhans cell
histiocytosis. Am J Neuroradiol 2004;25
: 880-891[Abstract/Free Full Text]
- Makras P, Samara C, Antiniou M, et al. Evolving radiological
features of hypothalamo-pituitary lesions in adult patients with Langerhans
cell histiocytosis (LCH). Neuroradiology2006; 48:37
-44[CrossRef][Medline]
- Max MB, Deck MD, Rottenberg DA. Pituitary metastasis: incidence in
cancer patients and clinical differentiation from pituitary adenoma.
Neurology 1981;31
: 998-1002[Abstract/Free Full Text]
- Brat DJ, Scheithauer BW, Staugatis SM, Holtzman RN, Morgello S,
Berger PC. Pituicytoma: a distinctive low-grade glioma of the neurohypophysis.
Am J Surg Pathol 2000;24
: 362-368[CrossRef][Medline]
- Fuller CE, Frankel B, Smith M, et al. Suprasellar monomorphous
pilomyxoid neoplasm: an ultrastructural analysis. Clin
Neuropathol 2001; 20:256
-262[Medline]
- Harnsberger H. Head and neck digital teaching
file. Salt Lake City, UT: Amirsys, 2002

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