KA, 69 yo male, complaining of back
pain, with recent urinary retention, gait problems. MRI discloses an extradural
mass compressing the medulla.
KA, patient de 69 ans admis pour compression
mdullaire D4-D5. Plainte depuis 1 an de dorsalgie interscapulaire s'tendant
aux deux membres infrieurs sans territoire radiculaire prcis avec des
zones de paresthsies. Troubles sphinctriens et de la marche rcents.
L'IRM dorsale montre un processus
occupant de l'espace extradural en regard de D4-D5 postro-latral gauche
comprimant la moelle, et sortant par le trou de conjugaison correspondant.
Sections show an angiomatous
pattern, with areas of hemorrhage.
The capillary structures
are lined by plump cells, with mitotic figures, and have papillary features
in some areas
Some papillary fronds
have a concentric hyaline core. Areas of hemorhage, show a fibrinous meshwork
marking the transition with the capillary structures.
At low power, some fragments
of the submitted material, are lined peripherally by large vessels.
In some of these
vessels, papillary structures are found, on deeper sections Trichrome
of the previous field.
endothelial hyperplasia in a probable angioma.
Intravascular papillary endothelial
hyperplasia is probably not a true neoplasm but is presented here because
of capacity to simulate microscopically benign and malignant vascular
tumors. First described by Masson in hemorrhoidal vessels as "vegetant
intravascular hemangioendothelioma", it is currently thought to represent
an exuberant, organization and recanalization of a thrombus, an interpretation
supported by immummohistochemical studies. It occurs in previously normal
vessels or in varices, hemorrhoids, hematomas, pyogenic granulomas, hemangiomas,
and angiosarcomas. The de novo ("pure") form is usually found
in the extremities (particularly the fingers) and the head and neck region,
whereas the type engrafted or pre-existing vascular disorder ("mixed")
tends to be in the trunk. It simulates angiosarcoma because of the presence
of papillary formations, anastomosing vascular channels, plump endothelial
cells. It is identified because of the exclusively intravascular nature
of the process, the lack of necrose bizarre cells, and atypical mitoses;
the characteristically fibrinous and/or hyaline appearance of the papillary
stalks; and frequent finding of residual organizing thrombi.
(Ref. Ackerman's surgical
pathology, 8th ed.)
about papillary endothelial hyperplasia in the nervous system:
Neurosurgery 1999 Dec;45(6):1478-80
Intravascular papillary endothelial hyperplasia causing cauda equina
compression: case report.
Taricco MA, Vieira JO Jr, Machado AG, Ito FY
Department of Neurosurgery, University of Sao Paulo School of Medicine,
Hospital, SP, Brazil.
OBJECTIVE AND IMPORTANCE: Intravascular papillary endothelial hyperplasia
(Masson's vegetant hemangioendothelioma) is a rare condition affecting
the neuraxis. In the literature, only one case of this lesion involving
the vertebral canal with spinal cord compression has been reported. We
present a case of cauda equina compression due to this abnormality.
A 17-year-old boy was admitted at our hospital with pain, numbness, paresis
of the left lower extremity, and bladder dysfunction of approximately
1 month's duration. Computed tomography and magnetic resonance imaging
of the spine revealed a tumor within the spinal canal at the T12-L1 level.
INTERVENTION: The patient underwent a T12-L1 laminectomy. An epidural
red nodular tumor was visualized and totally resected. The findings of
the pathological examination were compatible with intravascular papillary
endothelial hyperplasia. At follow-up examination 1 month after the operation,
the patient had complete resolution of the pain, and the motor deficit
and bladder dysfunction had improved significantly.
CONCLUSION: This rare benign
vascular lesion may be clinically and histopathologically mistaken for
an angiosarcoma. Because the intravascular papillary endothelial hyperplasia
can be cured by complete surgical resection, it is important to distinguish
between these two lesions to avoid inappropriate aggressive treatment.
J Comput Assist Tomogr 1999
Intracranial papillary endothelial hyperplasia: occurrence of a case
after surgery and radiosurgery.
Hagiwara A, Inoue Y, Shakudo M, Wakasa K, Sato K, Ohata K, Daikokuya H,
Department of Radiology, Osaka City University Medical School, Japan.
Papillary endothelial hyperplasia (PEH) is considered a form of endothelial
proliferation rather than a true neoplasm and is usually located in the
skin or subcutis. We report a case of intracranial PEH that occurred after
surgery for glioma and subsequent radiosurgery. CT and MR revealed an
enhancing extra-axial mass located left posterolateral to the brainstem.
Intracranial PEH is rare; to our knowledge, development of an intracranial
PEH after surgery and radiosurgery has not been previously reported.
Acta Neurochir (Wien) 1997;139(9):883-6
Multiple intracerebral intravascular papillary endothelial hyperplasia.
Duong DH, Scoones DJ, Bates D, Sengupta RP
Department of Neurological Surgery, George Washington University Medical
Washington, DC, USA.
Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign
reactive lesion usually found in thrombosed subcutaneous blood vessels.
It uncommonly occurs in the central nervous system and may be mistaken
for a more malignant type of tumour such as angiosarcoma. We present a
first case of multiple IPEH occurring intracranially in a 51-year-old
woman. She developed neurological compromises secondary to the mass affect
of the haematoma arising from one of the lesions. Prompt surgical evacuation
of the haematoma stabilized her condition. Surgical treatment, pathological
findings, radiographic characteristics, and a review of the literature
J Neurosurg 1997 Mar;86(3):558-63
Intravascular papillary endothelial hyperplasia of the sellar region.
Report of three cases and review of the literature.
Kristof RA, Van Roost D, Wolf HK, Schramm J
Department of Neurosurgery and Neuropathology, University of Bonn, Germany.
Intravascular papillary endothelial hyperplasia (IPEH) is considered a
reactive proliferation of endothelium associated with thrombosis. The
occurrence of IPEH in the cranial cavity is exceedingly rare. In this
article, the authors report three cases of IPEH that originated from the
cavernous sinus and extended into the sellar contents. The lesions were
resected incompletely in two cases and completely in one case. The IPEH
in one of the patients was incompletely resected and exhibited further
growth on magnetic resonance imaging 3 months postoperatively; local radiation
therapy was instituted. This led to shrinkage of the lesion over an additional
follow-up period of 3.5 years. In a review of the literature, the authors
located seven other cases of intracranial IPEH. The authors conclude that
clinically symptomatic intracranial IPEH should be completely resected
whenever possible, because it can cause considerable morbidity and mortality
and because it is prone to progression or recurrence.