Raelson 2014

Review Article
Chiasmatic-Hypothalamic Masses in Adults: A Case Series and

Review of the Literature
Colin Raelson, BA, Gloria Chiang, MD
From the Department of Radiology, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, New York (CR, GC).
ABSTRACT
Chiasmatic-hypothalamic masses are commonly seen in children with neurofibromatosis Keywords: Magnetic resonance imag-
type 1 and often demonstrate a benign clinical course. These masses are, however, rare in ing, glioma, optic chiasm, hypothalamus.
adults and require tissue sampling for diagnosis. Here, we present four cases of chiasmatic-
Acceptance: Received July 21, 2013,
hypothalamic masses in adults with pathologically proven diagnoses. We also provide a and in revised form February 26, 2014.
review of the literature and propose a limited differential in this age group. Accepted for publication March 31, 2014.
Correspondence: Address correspon-
dence to Gloria Chiang, MD, 525 E 68th
Street, Box 141, New York, NY 10065.
E-mail: [email protected].
J Neuroimaging 2015;25:361-364.
DOI: 10.1111/jon.12132
Introduction rological examination and visual field assessment showed no

Masses involving the optic chiasm and hypothalamus are most focal deficits.
commonly seen in children and young adults with type 1 An MRI of the brain showed two heterogeneously enhanc-
neurofibromatosis (NF1). These masses usually represent low- ing masses centered in the hypothalamus and left temporal
grade gliomas with a benign course, are often asymptomatic lobe, extensive associated T2 hyperintensity extending into the
at the time of diagnosis, and may undergo spontaneous regres- optic chiasm and tracts, suggestive of nonenhancing tumor and
sion in the absence of therapy.1 Even in the absence of NF1, vasogenic edema (Fig 1A, B). Differential considerations in-
chiasmatic-hypothalamic masses in young adults tend to show cluded multifocal glioma or metastatic disease. Partial resection
benign pathology, with pilocytic astrocytoma being the most demonstrated glioblastoma multiforme. The patient was then
common.2 started on concurrent temozolomide chemotherapy and radia-
In adults, chiasmatic-hypothalamic masses are rare and tion, followed by maintenance temozolomide, per institutional
demonstrate more aggressive behavior.3,4 We present a case protocol.
series of 4 adult patients with such masses and their corre-
sponding pathologically proven diagnoses. We then provide a Patient 2
review of the relevant literature in adults and propose a limited
A 62-year-old woman with no known medical history presented
differential that may be considered in this age group.
with acute vision loss, with an initial clinical suspicion of pitu-
itary apoplexy. An MRI of the brain demonstrated an enhanc-
Methods ing mass involving the hypothalamus and optic chiasm, with
Four adult patients with chiasmatic-hypothalamic masses iden- T2 hyperintensity along the optic tracts (Fig 2A, B).
tified by magnetic resonance imaging (MRI) between May 2012 A chiasmatic-hypothalamic glioma was favored; surgical re-
and May 2013 were included in this case series. To assess the lit- section demonstrated metastatic adenocarcinoma. Subsequent
erature, we performed a MEDLINE search through the United pelvic ultrasound demonstrated findings suggestive of an ovar-
States National Library of Medicine’s PubMed online database, ian neoplasm.
using the search terms: glioma, hypothalamus, optic pathway,
chiasm, and adult. Relevant case reports, reviews, and their Patient 3
references were also examined for further sources.
A 77-year-old woman with no known medical history presented
with blurry vision, nausea, and fatigue. After initial admis-
Case Series
sion for electrolyte management, she demonstrated cognitive
Patient 1
changes and a brain MRI was ordered.
A 70-year-old man with an extensive cardiac history presented An MRI demonstrated a enhancing suprasellar mass cen-
with expressive aphasia and progressive difficulty writing. Neu- tered in the hypothalamus/optic chiasm, with T2 hyperintensity
Copyright ◦ 2014 by the American Society of Neuroimaging

C 361
Fig 1. Axial (A) and sagittal (B) T1 gadolinium-enhanced images demonstrate heterogeneously enhancing masses centered in the left
temporal lobe and optic chiasm/hypothalamus.
Fig 2. Coronal T1 gadolinium-enhanced (A), coronal T2-weighted (B), and sagittal T1 gadolinium-enhanced (C) images demonstrate an
avidly enhancing mass involving the optic chiasm and hypothalamus, with vasogenic edema extending posteriorly along the optic tracts.
involving the optic chiasm, tracts, and internal capsules (Fig 3A- tinued decline in visual function. Finally, a biopsy demonstrated
C), suggestive of a glioma. Diffuse large B-cell lymphoma was an inflammatory neuritis with numerous acid-fast bacilli, iden-
confirmed on pathology. tified as Mycobacterium haemophilum on subsequent PCR (poly-
merase chain reaction) analysis. After treatment with rifabutin,
Patient 4 moxifloxacin, and azithromycin, the patient’s vision improved
A 44-year-old man with a history of HIV and a recent history and MRI findings resolved.
of CMV retinitis, status post ganciclovir treatment, presented to
an ophthalmologist with a week of visual loss. Ophthalmologic
examination was concerning for a central nervous system pro- Discussion
cess; the patient was sent to the emergency room for empiric Chiasmatic-hypothalamic gliomas account for a small portion
treatment for fungal and viral infection. (3%-5%) of all pediatric brain tumors, but they are seen in 11%-
A brain MRI demonstrated an enhancing mass involving 30% of children with NF1.5 Ninety percent of these tumors
the optic chiasm and hypothalamus, with T2 hyperintensity ex- occur in patients less than 19 years of age.6 These gliomas
tending along the left optic tract (Fig 4A, B), highly concerning tend to be nonaggressive low-grade tumors, with pilocytic as-
for a chiasmatic-hypothalamic glioma; lymphoma was also con- trocytoma being the most common pathology in young adults
sidered. Blood cultures and CSF (cerebrospinal fluid) sampling with and without NF1; Jahraus and Tarbell7 reported 5-year
were unrevealing. The patient remained empirically on anti- progression-free survival and overall survival rates of 72% and
fungal and antiviral medication and received doses of steroids, 90%, respectively. Varan et al1 found an even higher 10-year
IVIG (intravenous immunoglobulin), and penicillin, with con- progression-free survival rate of 76.5% in an NF1 population.
362 Journal of Neuroimaging Vol 25 No 3 May 2015

Fig 3. Axial T2 FLAIR (A,B) and sagittal T1 gadolinium-enhanced (C) images demonstrate an enhancing mass involving the optic chi-
asm/hypothalamus with associated T2 hyperintensity.
Fig 4. Coronal T1 gadolinium-enhanced (A), coronal T2-weighted (B), and sagittal T1 gadolinium-enhanced (C) images demonstrate a
T2-hyperintense, enhancing mass involving the optic chiasm/hypothalamus.
In the pediatric age group, particularly in the setting of to have multifocal glioblastoma multiforme, highlighting the
NF1, MRI findings associated with chiasmatic-hypothalamic importance of considering highly malignant tumors in this age
gliomas are generally pathognomonic, and biopsy is usually not group.
required. However, chiasmatic-hypothalamic masses in adults In addition to gliomas, we also present Patients 2 and 3
tend to have an aggressive clinical course, as demonstrated by in our series, who were found to have metastatic disease and
our case series, and do require tissue sampling for appropriate lymphoma involving the optic chiasm and hypothalamus. This
diagnosis and treatment. further corroborates the importance of considering other malig-
A review of the literature in this age group mirrored our nant etiologies in this age group and the need for tissue diagnosis
findings. Hoyt et al8 published the first case series of 5 adults for appropriate treatment.
patients who rapidly progressed to blindness over weeks and Only one other paper in our literature search reported infec-
death within months, coining the term “malignant optic glioma tions involving the optic chiasm and hypothalamus in adults.
of adulthood.” Subsequent cases reported by Wabbels et al,3 Bommakanti et al12 looked at 24 patients initially diagnosed
Dario et al,4 Dutton et al,6 Matloob et al,9 and Doreen with chiasmatic-hypothalamic gliomas on MRI, and 4 subse-
et al10 described a similar clinical course and surgical pathology quently were found to have tuberculomas on final pathology.
demonstrating glioblastoma multiforme or high-grade astrocy- Interestingly, Patient 4 in our case series was also found to have
toma. An analysis of 445 optic gliomas from the Surveillance, a mycobacterial infection that mimicked a mass.
Epidemiology and End Results (SEER) database of the National Therefore, based on our experience and the literature,
Cancer Institute, published by Mishra et al,11 also reported that we propose a limited differential diagnosis of a chiasmatic-
gliomas in the 5- to 19-year-old age group were 96% low grade, hypothalamic mass in an adult: (1) high-grade glioma, (2)
compared to only 22% in the 50+ age group. These reports are metastasis, (3) lymphoma, and (4) atypical infection. The imag-
consistent with Patient 1 from our case series, who was found ing findings may mimic the often self-limited glioma seen in
Raelson and Chiang: Chiasmatic-Hypothalamic Masses in Adults 363

children with NF1, with evidence of an enhancing mass, as- 5. Binning MJ, Liu JK, Kestle JRW, et al. Optic pathway gliomas: a
sociated vasogenic edema, and sometimes a component of review. Neurosurg Focus 2007;23(5):E2 1-8.
nonenhancing tumor. However, these masses in adults tend to 6. Dutton JJ. Gliomas of the anterior visual pathway. Surv Ophthalmol
1994;38:427-452.
have a more aggressive course. Finally, tissue sampling is often
7. Jahraus CD, Tarbell NJ. Review: optic pathway gliomas. Pediatr
needed to confirm the diagnosis and determining appropriate Blood Cancer 2006;46:586-596.
treatment. 8. Hoyt WF, Meshel LG, Lessell S, et al. Malignant optic glioma of
adulthood. Brain 1973;96(1):121-132.
References 9. Matloob S, Fan JC, Danesh-Meyer HV. Multifocal malignant optic
1. Varan A, Batu A, Cila A, et al. Optic glioma in children: a retro- glioma of adulthood presenting as acute anterior optic neuropathy.
spective analysis of 101 cases. Am J Clin Oncol 2013;36(3):287-292. J Clin Neurosci 2011;18:974-977.
2. Valdueza JM, Lohmann F, Dammann O, et al. Analysis of 20 10. Doreen L, de Oliveira FH, Bernays RL, et al. Rare suprasellar
primarily surgically treated chiasmatic/hypothalamic pilocytic as- glioblastoma: report of two cases and review of the literature. Brain
trocytomas. Acta Neurochir 1994;126:44-50. Tumor Pathol 2012;29:216-220.
3. Wabbels B, Demmler A, Seitz J, et al. Unilateral adult malignant 11. Mishra MV, Andrews DW, Glass J, et al. Characterizations and
optic nerve glioma. Graefe’s Arch Clin Exp Ophthalmol 2004;242:741- outcomes of optic nerve gliomas: a population-based analysis.
748. J Neurooncol 2012;107:591-597.
4. Dario A, Ladini A, Cerati M, et al. Malignant optic glioma of 12. Bommakanti K, Panigrahi M, Yarlagadda R, et al. Optic
adulthood: case report and review of the literature. Acta Neurol chiasmatic-hypothalamic gliomas: is tissue diagnosis essential? Neu-
Scand 1999;100:350-353. rology India 2010;58(6):833-840.
364 Journal of Neuroimaging Vol 25 No 3 May 2015

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Review Article

Chiasmatic-Hypothalamic Masses in Adults: A Case Series and

Introduction rological examination and visual field assessment showed no

Copyright ◦ 2014 by the American Society of Neuroimaging

362 Journal of Neuroimaging Vol 25 No 3 May 2015

Raelson and Chiang: Chiasmatic-Hypothalamic Masses in Adults 363

364 Journal of Neuroimaging Vol 25 No 3 May 2015

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