Turkis/i Neiirosiirgery 10: 54 - 57, 2000 Kiizey/i: Ma/igiiaiit [ibroiis /iistiocytoma

Primary OsseousOf The

MalignantSkull: A

Fibrous HystiocytomaCase Report

Kafatasinda Primer Osseöz Malign Fibröz HistiosItoma:Olgu Sunumu

KA YHAN KUZEYLI, ERTUGRUL ÇAKIR, ABDÜLKADIR REIS,

SÜLEYMAN BAYKAL, HA VVANUR TURGUTALP

Karadeniz Technical University Medical Faculty, Departments of Neurosurgery (KK, EÇ, SB) andPathology (AR, HT), Trabzon, Turkey

Received: 4.2.1999 ~ Accepted: 23.11.1999

Abstract: Malignant fibrous histiocytoma rarely affectsthe cranium. This report describes a case of primaryosseous malignant fibrous histiocytoma of the temporalbone in a 58-year-old male patient. The tumor was thoughtto have originated in the inner table of the bone of theskull, with subsequent intracranial-extracranial extension.Lung metastasis developed and the patient died 6 monthsafter the histopathological diagnosis. We discuss the caseand review the relevant literature.

Key words: Computerized tomography, magneticresonance imaging, malignant fibrous histiocytoma, skull

INTRODUCTION

Malignant fibrous histiocytoma (MFH) is amalignant tumor typically found in soft tissue andlong bones (4,6,9). This neoplasm has been reportedin areas of irradiated (ll) or traumatized bone, at sites

of infarcted bone (8), and in association with Pagefsdisease (7) and fibrous dysplasia (9), but MFH of theskull is extremely rare (2,4-6,9).

CASE REPORT

A 58-year-old male presented with a nonspecificleft temporoparietal headache. Physical examination

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Özet: Kraniyal malign fibröz histiosItomalar nadirtümörlerdir. Bu makalede 58 yasinda erkek hastadagörülen; temporal kemigi n malign fibröz histiositomaolgusunu sundu k. Tümörün iç tabuladan köken aldigi vedaha sonra intra ve ekstrakraniyal yayilim gösterdigidüsünüldü. Hasta cerrahiden alti ay sonra kaybedildi.Olgu ilgili literatür gözden geçirilerek tarhsildi.

Anahtar Kelimeler: Bilgisayarli tomografi, kafatasi,malign fibröz histiositoma, manyetik rezonansgörüntülerne

revealed pa in on palpation of the righttemporoparietal region as the only abnormality.The patienfs neurological exam was normaL.Cranial x-rays showed abnormal bone density in theright temporoparietal region (Figure 1).Computerized tomography (CT) revealedirregularity of the inner table of the skull bone inthis area, and a hyyperdense lesion with no masseffect in the adjacent brain tissue (Figure 2a). Abone window CT sean of the same lesion

showed marked destruction of the inner table

of the cranium at the site (Figure 2b).Contrast-enhanced CT seans revealed no changes inthe lesion's appearance. The patienfs blood

Tiirkis/i Neiirosiirgenj 10: 54 - 57, 2000

Figure 1: Lateral craniography shows abnormal bonedensity in the temporoparietal region.

bioehemistry and whole blood eount were normaL.A magnetic resonance imaging (MRI) study was alsoplanned.

Twenty days af ter initial presentation, thepatient's headaehe had worsened considerably.Examination revealed a palpable and painfulswelling in his right temporoparietal area. T2­weighted MR images showed a right calvarialextraaxial lesi on with intra-extraeranial extension

Figure 2a: Axial CT without contrast shows destructionof the inner table of the bone of the skull. Notethat there is no mass effect associated with the

hyperdense lesion in the adjacent parenchyma.

Kuzey/i: Ma/igiiniit fibroiis /iistiocytoma

Figure 3: T2-weighted axial MR! done 17 days af ter theCT sean shows rapid tumor growth. Theneoplasm is extraaxial and iso-hypointensecompared to the brain parenchyma, and a masseffect is now apparent.

(Figure 3). We performed a right-sidedtemporoparietal cranieetomy, and found a tumororiginating from the inner table of the bone of theskull, with no macroscopic evidence of duralinvasion. The mass was nodular and encased in athin capsule. Surgery involved total excision of the

Figure 2b: A bone window CT sean of the same lesionshows marked destruction of the inner table ofthe skull bone.

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Turkis/i Neurosurgery 10: 54 - 57, 2000

tumor, with no cranioplasty at the site of the bonedefect.

Histopathologic examination revealed that themass consisted of highly pleomorphic cells withatypical mitotic activity (Figure 4a-c).

Immunohistochemical studies showed that

the neoplastic cells stained strongly for vimentinand alpha-l-antichymotrypsin (Figure 4d), showedfocal mild staining for Factor 8 and 5-100 protein,and did not stain for epithelial membran eantigen, or smooth muscle adin and desmin. Thediagnosis was MFH, and the patient underwenttreatment with fractionated radiotherapy(4,500 cGy). The patient died 6 months af terthe histopathological diagnosis, due to lungmetastasis.

Figure 4a: A photomicrograph shows the tumor'scharacteristic storiform pattern. (HE xlOO)

Figure 4b: A photomicrograph shows pleomorphic tumorcells with atypical mitotic figures, as well asinfiltrating inflammatory cells. (HE x 400).

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Kuzey/i: Mn/ignniit fibrous /iistiocytomn

DISCUSSION

MFH is a malignant pleomorphic mesenchymaltumor that consists of a bicellular population offibroblasts and histiocytes in varying proportion s(2). This neoplasm is the most common malignanttumor of soft tissue (9,10,12), but is rarely seen in thebones of the skull and face (2,4-6,9,11). Our literaturesearch revealed only 20 cases of the latter type 0,3­5,9).

According to Nakayama et aL, MFH canoriginate from the innet table of the bones of the skull,from the diploe, or from the dura mater (9).Like otherskull tumors, MFH can present as painful soft-tissueswelling. The tumor in our case first destroyed theinner table of the bone in the patient' s righttemporoparietal area. At the time of our initial work­up, there was no notable intracranial or extracranial

Figure 4c: A photomicrograph shows a field denselypopulated with giant turnoral cells. (HE x200)

Figure 4d: Immunohistochemical staining reveals apositive reaction to alpha-l-antichymotrypsinin the cytoplasm of the tumor cells. (x 400)

Tiirkis/i Neiirosiirgery 10: 54 - 57, 2000

mass lesion (Figures 2a,b); however, MRI done 2weeks later revealed a significant intracranial­extraaxial mass lesion (Figure 3). The literatureprovides no detailed information about the rate atwhich these tumors grow, but theyare thought toprogress rapidly. Treatment for MFH involvessurgical removal followed by adjuvantchemotherapy and radiotherapy. Aggressivetreatment is vital because of the tumor's potential togrow and metastasize quickly (9).

In summary, this report is significant becauseprimary skull MFH is a highly unusual finding.Although there have been approximately 20 reportedcases to date, our report is the first to document theoccurrence of early bone destruction with noassociated mass effect. We believe that primary MFHof the cranium originates from the inner table of thebones of the skull rather than the dura mater. Our

case demonstrates the speed, at which these tumorscan grow, a threat that highlights the need for newtreatment modalities. The surgeon should always besuspicious of metastasis whenever a patient isdiagnosed with MFH.

Correspondence: Doç.Dr. Kayhan Kuzeyli,KTÜ Tip Fakültesi Nörosirurji A.B.D.61080, Trabzon, TurkeyTel: +90 462 377 5454Fax: +90 462 325 0518

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