ANNALS OF CLINICAL AND LABORATORY SCIENCE, Vol. 24, No. 4Copyright © 1994, Institute for Clinical Science, Inc.

Masson’s Hemangioma of Spinal Meninges Causing Cord Compression with Paraplegia*SYED Z. ALI, PETER M. FARMER, KAREN BLACK, and ALAN ROSENTHAL

Division o f Surgical Pathology, Department o f Laboratories,Department o f Radiology,

Division o f Neurosurgery, Department o f Surgery, North Shore University H ospital-

Com eli University Medical College, Manhasset, NY 11030

ABSTRACTMasson’s hemangioma is a rare benign pathological entity. A unique case

is reported of a 42 year old man with a spinal epidural mass who developed acute cord compression resulting in sudden onset of paraplegia. Histo­logical examination of the resected mass revealed a cystic vascular lesion associated with papillary endothelial cell proliferation, and overlying thrombosis, consistent with Masson’s hemangioma. Clinicoradiological correlation is also presented.

Introduction“Masson’s hemangioma” or “intravas-

cular papillary endothelial hyperplasia” is a rare pecu liar vascular lesion of benign nature. It was first reported by Pierre Masson in a case of inflamed hem­orrhoidal plexus1 who named it “intra- vascular vegetating hemangioendothe­liom a.” D esp ite its w ell d escribed histologic features, the exact histogenesis of this rare entity is still controversial.2,3 Skin and soft tissue are the most common site of this lesion,3 though it has been re p o r te d in th e oral m ucosa 4 and

* Address rep rin t requests to: Peter Farmer, M.D., D epartm ent of Laboratories, North Shore University H ospital-Cornell University Medical College, 300 Community Drive, Manhasset, NY 11030.

brain.5,6,7,8 A unique case is described of a 42-year-old male who had a spinal epi­dural mass which caused acute cord com­pression resulting in sudden paraplegia. This, to our knowledge, is the first case of Masson’s hemangioma at this site with an acu te and m ost u n u su a l c l in i ­cal presentation.

Case ReportA 42-year-old man was admitted to North Shore

University Hospital w ith two weeks history of severe upper abdominal pain radiating around from the back in the thoracolumbar region. In the past, he had been evaluated for lumbar disc disease. He consulted a chiropractor who m anipulated the spine, but without any improvement. He developed poor control of his left foot and a belt-like pain sub- costally followed by a gradual weakness of both legs which progressed to complete paraplegia. There was a remote history of head trauma secondary to a motor vehicle accident requiring craniotomy and

3710091-7370/94/0700-0371 $00.90 © Ins titu te for C lin ical Science, Inc.

372 ALI, FARMER, BLACK, AND ROSENTHALtemporary tracheostomy. Physical examination at the time of admission revealed anesthesia below L2 level bilaterally, increased tone in leg muscles and mid to lower thoracic spine tenderness. Magnetic resonance im aging (MRI) revealed an epidural mass at T 8 level causing marked cord compression. He underwent surgery with removal of a 1.5 cm mass associated with a hematoma. Postoperative recovery was satisfactory with gradual improve­ment of his paraplegia to paraparesis. He was sub­sequently discharged to a rehabilitation center for further care.

Ra d io l o g ic F in d in g s

Magnetic Resonance Imaging using Tj and T2 weighted images demonstrated a focal mass with nonspecific soft tissue signal, dorsal to the cord at the T8 level. The mass is epidural in location, caus­ing marked cord compression. Its signal character­istics are nonspecific and could represent acute hem orrhage, in fec tio n (abscess) or neoplasm (figures 1,2).

P a t h o l o g ic F in d in g s

Grossly, the specimen consisted of a soft, ovoid mass measuring 1.5 cm in greatest dimension.

FIGURE 2. Magnetic resonance imaging scan. T he m ass b eco m es b r ig h t on T 2-w e ig h te d image (arrowhead).

Histologically, the tumor was comprised of a vas­cular mass with a well-formed fibrous capsule sepa­rating it from the surrounding fibroconnective tis­sue, which in places, resem bled portions of a sclerotic vessel wall. Projecting within the cystic vascular mass were multiple papillary tufts formed by exuberant proliferation of plump, round endo­thelial cells. These papillary projections were pre­dominantly seen in a framework formed by a large organizing thrombus (figure 3).

Another pattern of endothelial proliferation was also noted in which the papillary projections were more delicate with or without fibrovascular cores and hyalinized stroma and were seen projecting into cavernous spaces of varying sizes filled with fibrin or disintegrating red cells (figure 4). The pro­liferating endothelial cells had plump, round to oval nuclei frequently with micronucleoli and had broad pink cytoplasm with well-defined cell membranes. No anaplasia or pleom orphism was noted, and mitotic figures were rarely present. Similarly, solid areas of endothelial proliferation and necrosis were absent, though in some fields, the lesion did show prominent piling up and stratification. Based upon these histologic findings, diagnosis of a Masson’s hemangioma was made.

FIGURE 1. Magnetic resonance imaging scan. Sagittal Tj-weighted image of the thoracolumbar spine demonstrates a soft tissue epidural mass pos­terior to the cord at Tg, causing cord compres­sion (arrowhead).

DiscussionThe list o f etio logic factors causing spi­

nal cord com pression and paraplegia is

MASSON’S HEMANGIOMA 3 7 3

long and in clud es both neoplastic and non-neoplastic processes. M asson’s hem ­angioma presenting as a spinal epidural mass is a unique clin ical and pathologic finding. This lesion o f vascular endothe­lia l o r ig in h as fa s c in a te d p a th o lo ­gists for years and has b een the topic o f much d iscussion esp ecia lly in regards to its doubtful neoplastic nature. Masson, w h en first describing this le s io n ,1 con­c lu d ed th e p a p illary p ro lifera tion o f endothelial ce lls as the primary process with fibrin deposition and thrombosis as a secondary phenom enon. This was chal­le n g e d la ter b y o th er au th o rs2 w h o b e liev ed that throm bosis p reced es the endothelial proliferation and, in fact, pro­vides an initial nidus or core for the pro­liferating endothelia l tufts. Light micro­scopic features o f this tumor are quite

F ig u r e 3. M asson’s hemangioma. Organizing thrombus with em bedded tufts of proliferating vas- c u l a r e n d o t h e l i u m . ( H e m a t o x y l i n a n d Eosin, x200)

characteristic and have been w ell studied by various authors2,3,9 and are essen tia lly similar to our case.

M asson’s hem angiom a is typ ica lly a small lesion with a slight female predomi­nance, a m edian age o f 39 years and a p red ilec tio n for fingers o f hands and head and neck area.3 T hese are typically s lo w g ro w in g le s io n s w ith e x c e l le n t prognosis, rare in ciden ce o f recurrence and n o m e ta s ta s is f o l lo w in g s u r g i­cal rem oval.2

D ifferential diagnosis of this unusual en tity can p resen t d ifficu lt prob lem s, e sp e c ia lly in regard to an giosarcom a and has b e e n a d d ressed b y va rio u s authors,3,10,11 som e o f whom ev en have suggested the term “M asson’s pseudoan­giosarcom a”3 to em phasize the h istologic sim ilarities o f the two conditions and the

374

F i g u r e 4 . M a s s o n ’s h e m a n g io m a . A c a v e r n ­o u s sp a c e w ith p a p i l la ry e n d o t h e l i a l p r o je c t io n s sh o w in g a d e l ic a te f ib ro - v a s c u l a r c o r e . ( H e m a ­to x y lin a n d E o s in , x 2 0 0 )

critical importance o f an accurate diag­n osis in th is s itu a tion . T h ere are no set gu id elin es for separating these two entities, h ow ever an intraluminal loca­tion o f the pap illary en d o th e lia l pro­liferation , a sso c ia ted w ith throm botic m ateria l and th e norm al e n d o th e lia l lin in g o f the in v o lv ed vascular space points to M asson’s hem angiom a.2 Pyo­genic granuloma is another important dif­ferential diagnosis esp ecia lly the atypical on es a lso k n ow n as p se u d o p y o g e n ic g r a n u lo m a .12 H o w e v e r , p r o m in e n t in fla m m a to ry c o m p o n e n t in c lu d in g

eosinophils and a haphazard endothelial proliferation w ithout formation o f anasto­m osing channels w ould point to the lat­ter lesion .3

In c o n c lu s io n , a u n iq u e c a s e is reported o f a spinal epidural mass, diag­nosed histologically as M asson’s hem an­gioma and presented clin ically as para­plegia. This rare entity should be kept in mind in differential diagnosis o f vascular le s io n s , e sp e c ia lly angiosarcom a, and sh ou ld b e id en tified as a co m p le te ly benign lesion am enable to sim ple surgi­cal excision.

ALI, FARMER, BLACK, AND ROSENTHAL

MASSON’S HEMANGIOMA 3 7 5Acknowledgment

Thanks are extended to Ms. Mei Wu for her excel­lent secretarial assistance.

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4. Heyden G, Dahl I, Angervall L. Intravascular papillary endothelial hyperplasia in the oral mucosa. Oral Surg 1978;45:83-7.

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9. Kreutner A Jr, Smith RM, Trefney FA. Intravas­cular papillary endothelial hyperplasia. Light and electron microscopic observation of a case. Cancer 1978;42:2304-10.

10. Barr RJ, Graham JH, Shermin LA. Intravascular papillary endothelial hyperplasia. A benign lesion mimicking angiosarcoma. Arch Dermatol 1978;114:723-6.

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12. W ilson-Jones E, B leehan SS. Inflammatory angiomatous nodules with abnormal blood ves­sels occurring about the ears and scalp (pseudo or atypical pyogenic granuloma). Br J Dermatol 1969;81:804-16.