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Multifocal osteoclastoma of the skull: case report

Osteoclastoma craniano multifocal: relato de caso

Abstracts

We describe the case of a 35 years old man with a nonspecific complaint of a slow growing solid mass in the frontal region. Radiological exams evidenced two more lesions : in the superior and lateral walls of the orbit. Treated with total excision of the lesions and a cranioplastic procedure at the same act, with favorable outcome. Microscopic findings suggested giant cell tumor in the three lesions that was confirmed by imunohistochemical examination.

osteoclastoma; giant cell tumor; multifocal; skull


Descrevemos o caso de homem de 35 anos, com história de lesão endurecida de crescimento progressivo na região frontal direita. Exames radiológicos evidenciaram duas outras lesões: uma na parede superior e outra na parede lateral da órbita direita. Submetido a exérese total das lesões com margem de segurança e cranioplastia no mesmo tempo cirúrgico. O exame anatomo patológico sugeriu tumor de células gigantes nas três lesões, diagnóstico confirmado pela imuno-histoquímica.

osteoclastoma; tumor de células gigantes; multifocal; crânio


Multifocal osteoclastoma of the skull: case report

Osteoclastoma craniano multifocal: relato de caso

Ricardo Souza QuadrosI; Atos Alves de SousaII; Gervásio Telles Cardoso CarvalhoIII; Marcos Antônio Dellaretti FilhoI

INeurosurgery Resident, Santa Casa de Belo Horizonte

IIM.D.,PhD., Professor at the Department of Neurosurgery, FCMMG

IIIM.D., Professor at the Department of Neurosurgery, FCMMG

ABSTRACT

We describe the case of a 35 years old man with a nonspecific complaint of a slow growing solid mass in the frontal region. Radiological exams evidenced two more lesions : in the superior and lateral walls of the orbit. Treated with total excision of the lesions and a cranioplastic procedure at the same act, with favorable outcome. Microscopic findings suggested giant cell tumor in the three lesions that was confirmed by imunohistochemical examination.

Key words: osteoclastoma, giant cell tumor, multifocal, skull.

RESUMO

Descrevemos o caso de homem de 35 anos, com história de lesão endurecida de crescimento progressivo na região frontal direita. Exames radiológicos evidenciaram duas outras lesões: uma na parede superior e outra na parede lateral da órbita direita. Submetido a exérese total das lesões com margem de segurança e cranioplastia no mesmo tempo cirúrgico. O exame anatomo patológico sugeriu tumor de células gigantes nas três lesões, diagnóstico confirmado pela imuno-histoquímica.

Palavras-chave: osteoclastoma, tumor de células gigantes, multifocal, crânio.

Osteoclastoma or giant cell tumor is a benign but often locally aggressive neoplasm of the bone. It occurs more frequently between the third and fifth decades of life, sometimes after patients are fifty years old and rarely after they are sixty years old. This is one of the few osseous neoplasms showing higher occurrence in women1-3. Giant cell tumor accounts for about 5 % of biopsed primary bone tumors and about 20% of benign bone tumors. Most of the osteoclastomas are located near the articular end of tubular bones. The skull is rarely involved, only 1 to 2% of the cases described in the literature3-6. We describe a case of osteoclastoma.

CASE

A 35 year-old white man presented with a 1 year history of a slow growing solid mass at the right frontal region with about 2 cm of diameter. The lesion was surgically resected in another hospital, but probably partially because 4 months later it was back. The CT scan at our service showed two other lesions in the skull: in the superior and lateral walls of the right orbit (Figs 1 and 2). All the three lesions were completely resected and a cranioplastic procedure was performed at the same act. Microscopic findings suggested giant cell tumor in the three lesions, and that was confirmed by imuno-histochemical exams (Fig 3).




DISCUSSION

Giant cell tumor or osteoclastoma is a rare tumor, representing only 3 - 7% of all bone tumors. About 70 - 90% are located at or close the extremities of long bones, 10 - 30% are located in the sacrum, region of the knee, small bones of the hands and vertebras, 2% affect the skull, more commonly the mandible and maxilla1,2,7,8. A review of the literature demonstrated 7 series of giant cell tumors totalizing 2404 cases1,2,6-10, and 24 (1%) occurred in the skull, excluding the ones found in the mandible. When located in the skull they are more frequently found at the sphenoid and temporal bones, and rarely at the ethmoidal, frontal or occipital bones5.

Multifocal osteoclastoma is rare, usually occurring in the hands and feet10. The multifocal osteoclastoma should not be confused with giant cell reparative granuloma, which is often multifocal and presents a high incidence of recidivation. In an extensive review of the literature no other case of multifocal osteoclastoma of the skull was found, and we believe this is the first published case. The most common radiological appearance of osteoclastoma in the skull is an expanding and/or lytic neoplasm that may appear to extend into the surrounding soft tissues, dura or sinuses. The rarity of these lesions explains the few numbers of MRI and CT descriptions of skull giant cell tumors7,11.

The presence of giant cells in the histochemical study does not confirm the diagnosis. The differential diagnosis includes: giant cell reparative granuloma, benign fibrous histiocytoma and osteosarcoma with prominent giant cells6. The most common macroscopic appearance of osteoclastoma is a grayish, soft lesion with small cysts and occasional necrotic areas. Our patient presented in the microscopic exam: multinucleated giant cells, mononuclear cells and few histiocytes with hemossiderin deposition. The imunohistochemical study confirmed CD68, a bone giant cell marker.

More than 60% of giant cell tumors will recidivate if treated with partial resection. In addition to local intraosseous recurrence, surgery-related contamination may lead to implantation of cells of the tumor in the perilesional soft tissues in 10 - 15% of patients. The use of radiotherapy should probably be limited to lesions that cannot be subjected to total excision, and that is an issue for further literature discussion12-15.

Received 27 December 2002, received in final form 27 July 2003

Accepted 5 September 2003

Neurosurgery Department, Santa Casa de Belo Horizonte/Faculdade de Ciências Médicas de Minas Gerais (FCMMG), Belo Horizonte MG, Brazil

Dr. Ricardo Souza Quadros - Rua Monte Sião 212/302 - 30240-050 Belo Horizonte MG - Brasil.

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Publication Dates

  • Publication in this collection
    28 Apr 2004
  • Date of issue
    Mar 2004

History

  • Reviewed
    27 July 2003
  • Received
    27 Dec 2002
  • Accepted
    05 Sept 2003
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