Tllrkish Nellrosllrgery 12: 120 - 123, 2002 Knynsd(llk: Lllmbosncral Mellillgelll Hnl1wrfomn Awl Hlfperfrichosis

Lumbosacral Meningial Hamartoma And Hypertrichosis:A Case Report

Hipertrikozisli Lumbosakral Meningial Hamartoma:Bir Olgu Sunumu

FAZiLET KA YASELC::UK,DERYA GDMDRODLD, SUZAN ZORLUDEMiR, MAHMUT DOGAN AY

Pathology Department, Ba~kent University Faculty of Medicine (FK), Pathology Department, (ukurova UniversityFaculty of Medicine (DG), Pathology Department, (ukuwva University Faculty of Medicine (5Z), Department of

Neurosurgery, Adana Government Hospital (MD)

Received: 13.07.2001 ~ Accepted: 28.01.2002

Abstract: Objective: Ectopic meningiomas are rare tumors.In this paper, we describe a patient with lumbosacralmeningeal hamartoma and hypertrichosis.Methods: An 18-year-old female with a red-violet skinlesion on her lower back was determined spina bifida atL5-51 by x-ray. Magnetic resonance imaging sequencesshowed a fibrous band arising from a posterior fusiondefect at L5-51. Light microscopy examination of thespecimen were seen islets of meningothelial cells andcalcified psammoma bodies spread among the vessels,neuronal structures, and dense collagenous tissue.Result: We present a case lumbosacral ectopic hamartomawith hypertrichosis and a review of the nomenclature,classification and pathogenesis of this disorder.Cone/usiol/: Ectopic meningiomas are rare tumors in whichetiopathogenesis is not clear. They are usually independentof intracranial meningiomas. These lesions characterizewith clinical and pathological diagnostic difficulties. In theliterature, these lesions have been given a lot of mImeslike ectopic meningioma, meningial hamartoma, cutenousmeningioma. Because this lesion did not consist solelyof meningial elements, we suggested tha t the cases shouldbe more accurately labeled as meningial hamartoma.

Key Words: Meningial hamartoma, hypertrichosis,lumbosacral region

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Ozet: Allla~: Ektopik meningiomalar nadir gortilentLimorlerdir. Bu <;ah~mada hipertrikozisli, lumbosakralyerle~imli bir ektopik hamartoma olgusu sunulmu~tur.Metod: 51rtmda klrmlzl-mor cilt lezyonu olan 18 ya~mdabayan hastada direct grafide L5-51 de spina bifidasaptandl. Magnetik rezorans incelemesinde L5-S1 dekiposterior fLizyon defektinden kaynaklanan fibroz bir bantgortildLi. Lezyonun 1~lk mikroskopik incelemesindedamarlar, sinirler ve yogun kollojen doku boyunca uzananmeningotelyal hLicre adalan ve kalsifiye psammomacisimleri gori.ildLi.SOIllI~: Biz hipertrikozisli, lumbosakral yerle~imli birektopik meningeal hamartoma olgusu sunduk ve bulezyonlann isimlendirme, slluflama ve patogenezinigozden ge<;irdikYOn/III: Ektopik meningiomalar etyopatogenezi tam olarakbilinmeyen, nadir gorLilen tLimorlerdir. Genelliklemeningiomalarla ili~kisiz olarak bulunurlar. Bulezyonlarklinik ve patolojik tam zorluklan i<;erirler. Kaynaklardabu lezyonlara ektopik meningioma, meningialhamartoma, kutenoz meningioma gibi pek<;ok isimverilmektedir. Bulezyonlar sadece meningial elcmentlerii<;ermedigi i<;in biz meningial hamartoma olarakisimlendirmenin daha dogru oldugunu tavsiyeetmekteyiz.

Anahtar kelimeler: Meningial hamartoma, hipertrikozis,

lumbosakral bolge

Turkish Neurosurgery 12: 120 - 123, 2002

INTRODUCTION

Meningioma is the most common neoplasm ofthe central nervous system (CNS). It is believed thatthese tumors arise from arachnoid cap cells (1-3).Theyare rarely encountered in regions other than the CNS.Ectopic meningiomas, which account for 1-2% of allmeningiomas, most often develop in the scalp, the skinof other parts of the body, the subcutaneous tissue, thelungs, and the mediastinum. Those that arise in theskin or subcutaneous tissue have also been labeled

cutaneous meningioma 0,3,6-9,16-18).

CASE REPORT

An 18-year-old female with a congenital red­violet skin lesion on her lower back that included atuft of hair was admitted to the neurosurgery clinic atAdana Hospital. The patient's personal and familymedical histories were unremarkable, and herneurological examination and routine laboratory testswere all normal. Physical examination revealed a 6x10cm elliptical, circumscribed, red wine-colored lesionwith localized hypertrichosis. The lesion was locatedon the midline of the lower lumbar region. There wasno evidence of meningomyelocele. A lumbosacral x­ray demonstrated spina bifida at L5-Sl. Magneticresonance imaging sequences showed a fibrous band,arising from a posterior fusion defect at LS-Sl. Theband extended through the subcutaneous fat tissue inthis area, and appeared to be connected to a nodular

Figure 1: Magnetic resonance imaging shows the fibrousband arising from the posterior fusion defect atLS-Sl.

Kayasel~uk: LUII/bosacral Meuillgeal Hall/artoll/a Alld Hypcrtric1l(lsis

subcutaneous structure (figure 1). The subcutaneousnodular lesion was totally excised, with thesurrounding skin and the associated fibrous 'band,"which extended as a tract that communicated with the

spinal canal. The patient was discharged on the 3 rJ

postoperative day, and follow-up examinationrevealed no recurrence.

The surgical specimen was 6x6x4 cm, and ,·vascomposed of soft-tissue material covered by intacthairy skin. A firm mass of tissue with irregular borderswas localized deep inside the specimen. Lightmicroscopy examination showed areas of normalepidermis and dermis in the superficial parts of thespecimen. The deeper areas contained islets ofmeningothelial cells and calcified psammoma bodiesthat spread among the vessels, neuronal struchlres, anddense collagenous tissue (figure 2 a-b). Nopleomorphism, mitoses, or atypical cells were seen. Onimmunohistochemical study, the meningothelial cells

Figure 2 a: Islets of meningothelial cells and calcifiedpsammoma bodies between vessel and neuronalstructures in the subcutaneous tissue. (HEX40)

2 b: Group of meningothelial cells andpsammoma bodies in higher magnification(HEX400)

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Tl/rki,11 Nel/rosl/rgery 12: 120 - 123, 1002

stained positive for epithelial membrane antigen(EMA).

DISCUSSION

Ectopic meningiomas differ from intracranialmeningiomas regarding their associated clinical andhistopathological findings, and prognosis. In 1974,Lopez et al. reported a series of 25 cases that involvedmostly the scalp, neck, and back, but only one patienthad a lumbosacral cutaneous meningioma(7). Theseauthors have developed a system for classifyingcutaneous meningiomas in three different types (TableI). Type I is a congenital non-neoplastic lesion, and theother two types are forms of acquired neoplasticproliferation of meningothelial cells. Type I lesions, alsocalled primary cutaneous meningioma (PCM), aretypically misdiagnosed as alopecia, fibroma, or nevusin the clinical setting(7,14,18) PCMs have also beenfound in association with von Recklinghausen'sdisease, and a familial pattern has beenreported (1,7,12,18)

It is suggested that PCMs arise from arachnoidcap cells in the subcutis or cutis as a result of adevelopmental defect. Light microscopy study of thesetumors reveals masses of meningial cells that eithersurround the vessels or spread throughout a dense ormyxoid stroma. One PCM subtype that Lopez and co­workers labeled as "rudimentary meningocele" ischaracterized with a cystic cavity obliterated byfibrocollagenous proliferation and arachnoid cellhyperplasia (7,11,13,14,16,18). Forty-sevenrudimentary meningiocele cases had been reported inthe English literature until 1994. Listed from highestto lowest frequency of occurrence, these lesions werenoted in the scalp, vertebrae, forehead, and neck. Innine cases, the tumor was associated with a fibrous

tract that communicated with the CNS. Six patients

Table I: Classification of cutaneous meningiomas.

Kayaselql/k: Ll/l/llJosacml MCllillgclli Hal/lartol/la Alld HI/pcrtricilOsis

exhibited abnormalities of the bone underlying thelesion. The most common bone abnormality was spinabifida (1-18). Our patient's extracranial meningiomawas located in the lumbosacral region, which is a raresite of occurrence. There was no cystic cavity, but theyoung woman had spina bifida and there was a fibroustract extending from the tumor to the spinal canal.

Another variant of PCM, the "acoelic 111eningialhamartoma," has no cystic cavity, but hyperplasticmeningial cells and psammoma bodies are observedwithin the surrounding tissue as untidy foci (5,7,11,16).Suster and Rosai claimed that the cases that as acoelic

meningial hamartomas or rudimentary meningocelesin Lopez's classification should be labeled as "ectopicmeningial hamartomas"(15). Their rationale forincluding the word hamartoma was the presence of acombination of meningial cells, vessels, adipose tissue,and other mesangial cells(5, 13,15). In contrast to ectopicmeningial hamartoma, the cellular composition ofPCM is limited to meningial cells alone, but thesetumors also contain many psammoma bodies. Theakerand colleagues reported six cases of ectopicmeningioma characterized by meningothelial cells inthe vicinity of small nerves, and called these lesions"cutaneous heterotropic meningial nodules" (17).Hirakawa et al. suggested that meningial hamartomasshould be classified somewhere between meningoceleand PCM (5).

The clinical and histopathological findings in ourcase were compatible with type I PCM; however, wesuggest that, since this lesion did not consist solely ofmeningial elements, the label of meningial hamartomawould be more appropriate.

Regarding the localized hypertrichosis in ourpatient's lesion, we were able to find only one similarcase in the literature. In this case reported by Penas

Ori inA e of onsetLocation

CNSmenin ioma

Behavior

Histopathologicalfeatures

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Type I: Primarycutaneous menincon enitalchildhood

scalp, face, neck,aravertebral re ion

none

beni n

meningothelial cellproliferation similar toluenm lOma

Type II: Ectopic meningioma of thesoft tissue extendin to the skinac uiredadult

periorbital, perinasal,eriauricular

none

neo lastic

menmglOma

Type III: CNS meningiomaextend in to the skinac uiredadult

present

neo lastic

luenmglOma

Turkish Nellrosurgery 12: 120 - 123, 2002

and associates, the tumor was located in the

paravertebral region, but the patient did not have spinabifid a and there was no communicating tract(13).

To establish the definitive diagnosis in ectopicmeningioma, rCM must be histopathologicallydistinguished from peri neuroma, glomus,schwannoma, and Spitz nevus (2,3,6,10,15). Theimmunohistochemical and ultrastructural features of

ectopic meningiomas are different from these lesions.Like their intracranial counterparts, they also stainpositive for EMA and vimentin and stain negative forcytokeratin, 5-100 protein, desmin, neuron- specificenclose (NSE), chromogranin A. Also, this diagnosticstaining property supports the theory that these tumorsoriginate from arachnoidal cells (3A,1 US ). Glomustumors tend to be more vascular in nature and theyare immunohistochemically positive for chromogranin(6). Nevomelanocyctic and nerve sheat tumors(schwannoma, perineuroma) which are positive for5-100 protein can be distinguished by routine lightmicroscopy and immunohistochemical methods (2). Inour case in which a hair component was observed,Becker's nevus, familial hypertrichosis, and congenitalspinal cord hypertrichosis must also be included in thedifferential diagnosis (13). Congenital localizedhypertrichosis may be located away fom the spine andmay be associated with other cutaneous abnormalities(13). Our patient has a single cutaneous lesion on herback and her familial history was negative for familialhypertrichosis.

The prognosis is good for both forms of rCM,and Lopez et al. reported no deaths in their ectopicmeningioma type I cases during 20 years of follow-up(7). There is virtually no recurrence after appropriatesurgical resection. In our patient, postoperativerecovery was uneventfut and a follow-up examinationat 24 months revealed no recurrence or metastases.This case is unusual because of the lumbosacrallocalization of the lesion, and the fact that the lesion

exhibited hypertrichosis, and had a fibrous tract thatcommunicated with the spinal canal.

Correspondence: Dr. Fazilet Kayasel<;uk, Ba~kentUniversitesi, Adana Hastanesi,Yiiregirl ADANA, Tiirkiyee-mail: faziletks@yahoo.com

Kayaselquk: Lumbosacral MClIillgeal Hamartoma AI/d Hypcrtricho:,is

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