AJR 2005; 184:S10-S11
© American Roentgen Ray Society
Phrenic Nerve Hemangioblastoma in a von Hippel-Lindau Patient
Jesse Johnson,
Joseph Junewick and
Sandra Cottingham
Spectrum Health Hospitals, 100 Michigan NE, Grand Rapids, MI 49503.
Received January 4, 2004;
accepted after revision June 1, 2004.
Address correspondence to J. Junewick
(joseph.junewick{at}spectrum-health.org).
Introduction
Hemangioblastomas are benign, highly vascular tumors that
predominantly occur in the central nervous system. Thirty percent of
hemangioblastomas are associated with von Hippel-Lindau disease, an autosomal
dominant disorder also characterized by retinal angiomas, renal cysts and
carcinomas, pheochromocytomas, and pancreatic cysts and carcinomas
[1]. Hemangioblastomas occur
predominantly in the posterior fossa, spinal cord (< 5%)
[2], less commonly in the
brainstem, rarely supratentorially
[1], and rarer still
extradurally [2,
3]. Extradural
hemangioblastomas generally occur near the nerve roots, often widening the
exiting foramina [4]. Only two
other hemangioblastomas have been reported along a peripheral nerve in purely
extravertebral locations [2,
3].
Case Report
A 48-year-old woman with von Hippel-Lindau disease and a history of
previous multiple spinal and cerebellar hemangioblstomas complained of
soreness and swelling in the right side of her neck. CT
(Fig. 1A) and MRI (Figs.
1B and
1C) examinations were
performed. The mass was surgically resected from around the normally
functioning right phrenic nerve. Immunohistochemistry stained positively for
vimentin, neuron-specific enolase, and S-100, and negatively for cytokeratins
AE1/AE3 and epithelial membrane antigen, confirming hemangioblastoma.
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Fig. 1B. —CT of neck with contrast shows 3.5 x 2.8 cm
heterogeneously enhancing ovoid mass at level of larynx on right. Sagittal
T1-weighted image with gadolinium shows heterogeneous enhancement with few
scattered vascular flow voids.
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Discussion
Hemangioblastomas in various areas of the nervous system have shown fairly
consistent imaging characteristics despite their location
[1]. Hemangioblastomas are
cystic or solid or both. They appear well circumscribed, typically ovoid or
nodular, and, when larger than 15 mm, they may show vascular flow voids on MRI
and almost always do when larger than 24 mm
[4]. They rarely have
peritumoral edema [1,
4]. The solid component of
hemangioblastomas intensely enhances on CT and MRI, with slightly more
heterogeneous enhancement with an increase in tumor size
[4]. Unenhanced T1 signal
varies with the size of the solid component, showing homogeneous isointensity
(< 1.1 cm), hypointensity (1.1-2.0 cm), and variable signal intensity (>
2.0 cm) relative to surrounding neural tissue
[4]. T2 signal of the solid
component shows hyperintensity that becomes progressively heterogeneous with
increasing size [4]. With
unenhanced CT, the solid component of the hemangioblastoma is isodense to
surrounding neural tissue [1].
Cystic components of a hemangioblastoma are occasionally of slightly higher
density than cerebrospinal fluid on CT because of a slightly greater protein
content in the cyst fluid [1],
and isointense to slightly hyperintense to cerbrospinal fluid on T1, and
always hyperintense on T2
[1].
The hemangioblastoma in this case displayed the characteristic appearance
of a large hemangioblastoma, as did the two other reported cases of
extravertebral peripheral nerve hemangioblastomas
[2,
3]. Extravertebral peripheral
nerve hemangioblastomas seem to lack cystic components and tend to be large,
but otherwise have imaging characteristics similar to central
hemangioblastomas. The characteristic intense enhancement and signal flow
voids are clues to their vascular nature, but do not necessarily differentiate
them from other vascular neoplasms such as arteriovenous malformations,
paragangliomas, or metastatic renal cell carcinoma
[4].
References
- Iplikcioglu AC, Yaradanakul V, Trakya U. Supratentorial
haemangioblastoma: appearances on MR imaging. Br J
Neurosurg 1997;11:576
-578[Medline]
- Giannini C, Scheithauer BW, Hellbusch LC, et al. Peripheral nerve
hemangioblastoma. Mod Pathol1998; 11:999
-1004[Medline]
- Brodkey JA, Buchignani JA, O'Brien TF. Hemangioblastoma of the
radial nerve: case report. Neurosurgery1995; 36:198
-200[Medline]
- Chu BC, Terae S, Hida K, et al. MR findings in spinal
hemangioblastoma: correlation with symptoms and with angiographic and surgical
findings. AJNR2001; 22:206
-217[Abstract/Free Full Text]
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Introduction
Case Report
