Desmoplastic Fibroma of the Skull； ； ； ； A Case Report, Review of the Literature, and Therapeutic Implications
Joo Han Kim, M.D.,* Jung Yul Park, M.D.,* Yong Gu Jung, M.D.,*
Jung Keun Suh, M.D.,* Sung Nam Kim, M.D.,** Yeon Lim Suh, M.D.***
Department of Neurosurgery,* Medical College, Korea University, Seoul, Korea Greencross Clinical Center,** Seoul, Korea
Department of Pathology, Seoul Samsung Hospital,*** Medical College, Sungkyunkwan University, Seoul, Korea
= Abstract =
두개골에 발생한 결합조직형성 섬유종；증례보고 및 문헌 검토
고려대학교 의과대학 신경외과학교실,* 적십자 임상센터**
성균관대학교 의과대학 서울삼성병원 병리학교실***
김주한*·박정율*·정용구*·서중근*·김성남**·서연림***
esmoplastic fibroma(DF) is a rare neoplasm of the bone, and is histologically benign but locally aggressive disease. A total of nine cases of DF involving skull have been reported in the literature, and among these eight are females. In this report, the clinical findings and histopathology of a case with DF of the skull in a male patient is presented with a review of the literature with an emphasis on treatment modalities. A 21-year-old man presented with headache. CT scan revealed a solitary and lytic skull lesion without brain invasion. DF was confirmed by his- tological evaluation. On immunohistochemical staining of the tumor was negative for estrogen or progesterone receptors. After total resection of tumor with wide surgical margin, there was no recurrence during the 35 months of follow-up period. Although longer follow up period maybe needed, treatment of this type of tumor with complete resection of tumor tissue along with a wide margin may provide long disease-free state compare to the high recurrence rates in DF of other sites.
KEY WORDS：Desmoplastic fibroma・Parietal bone・Operative treatment.
Introduction
Desmoplastic fibroma(DF) is a rare bone tumor. It is histologically benign but locally aggressive intraosseous neoplasm that was initially described as a distinct clinico- pathological entity by Jaffe in 195811). It accounts for only nine of the 8542 osseous tumors reviewed at the Mayo Clinic5). Sites of predilection are mandible, long bones, and iliac bone9). Only 9 cases of DF have been reported invol- ving skull, all except one in female patients3)7)8)12)16-19).
In this report, we present the radiographic and histopa- thological findings of a case with DF in the right parietal bone of a male patient.
Case Report
A 21-year-old Korean male patient was initially evaluated for persistent right-sided headache that had lasted several weeks prior to admission. He had no history of head injury or other medical illnesses. Physical examination revealed a painful protruding mass on the right parietal region. Routine skull x-ray showed a round 4-cm lytic lesion in the right parietal bone without bone expansion or sclerosis(Fig. 1).
Computerized tomography(CT) demonstrated a lytic skull lesion with destruction of both skull tables(Fig. 2). The lesion had a central soft-tissue component without brain invasion.
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The skull lesion was completely removed along with wide margin and a cranioplasty was performed. Gross examin- ation of the specimen revealed a firm fibrous gray tumor between the internal and external tables of the skull. Mic- roscopic examination revealed that the lesion was composed of relatively uniform spindle-shaped fibroblasts with neo- plastic proliferation and with interspersed inflammatory cells(Fig. 3, Right). There was infiltrative growth of tumor tissue into the bone with entrapped sclerotic bony trabeculae (Fig. 3, Left). These findings were consistent with DF.
Immunohistochemical stains for estrogen and progesterone receptors were negative. There was no evidence of recurrence during the 35 months of follow-up period.
Discussion
Desmoplastic fibroma, a very rare benign tumor occurring predominantly in the metaphysis of long bones5). This case adds to the nine cases of DFs in the skull that have been previously reported and is the second case which occurred in a male patient(Table 1).
Bhm reviewed 171 cases of DF in 19962) and found that the numbers of male and female patients were equivalent with a mean age at presentation of 23 years. However, DFs that have been previously reported in the skull were predo- minantly in females with female to male ratio of 8：1 and the patients’ ages ranged from 7 years to 86 with a mean age of 36(Table 1). The parietal bone(4 cases) was the most frequently involved while three lesions were in the temporal bone and two in the frontal bone.
Triantafyllou et al. suggested a possible hormonal dep- endence of DF and reported a patient who showed a rapid proliferation of a recurrent tumor during pregnancy20). The desmoid tumors of soft tissue considered as nonosseous counterpart of DF were found to express estrogen and pro- gesterone receptors in up to two-thirds of the investigated patients1). In addition, one-half of the desmoid tumors of soft tissue responded to endocrine therapy21). For this reason, we performed immunohistochemical studies to verify the possible presence of estrogen and progesterone receptors.
Both were negative in our case.
Fig. 1. Skull anteroposterior(left) and lateral(right) x-ray showing a round 4-cm lytic lesion in the right parietal bone with nonsclerotic border.
Fig. 2. Left：Computerized axial tomography scans with a bone window showing a lytic area with uneven involvement of both tables. Right：A central soft-tissue component is seen without invasion of the brain.
Various skull lesions may radiographically mimic the appearance of DF15). There are no specific radiological fea- tures of DF in that a similar radiological findings can also be seen in other lesions such as eosinophilic granuloma, aneurysmal bone cyst, medullary fibrosarcoma, hemangioma, ameloblastoma, and metastatic tumors from the thyroid or kidney4). Thus, the final diagnosis requires a careful hist- ological examination.
Grossly, the tumor varies in color from gray to white and has a firm and rubbery consistency. Microscopically, DF is composed of mature fibrous tissue consisting of small, sparse fibroblasts with little or no mitotic activity in an abundant stroma of collagenous matrix2)6)10). This feature is important in histologically differentiating DF from low grade fibrosarcoma. The typical firbrosarcoma is more cellular with a herringbone pattern that shows more pleo- morphism and higher mitotic activity. However, in some cases of low grade fibrosarcoma, mitoses may not be evident and areas with predominant collagen tissue may be found, making the distinction from DF extremely difficult19).
In such cases, the final diagnosis can be established only after follow-up evaluation13). Because of the high recurrence rate of DF(about 20-30% of all cases) and local invasive- eness, complete resection with a wide margin is mandatory14). According to Pensak, hormonal regulation was considered effective in young patients and to date, early response to Tamoxifen appears to be beneficial although long-term evaluation is needed18). Previously reported cases of DF in the skull were treated by complete resection without adju- vant therapy. None of these cases showed recurrence unlike DFs of the long bones. The authors believe that this may be due to the feasibility of wide resection of skull lesions as compared to DFs of other sites.
Since our case was negative for immunohistochemical staining for estrogen and progesterone, no adjuvant therapy was warranted.
Conclusion
This case represents the tenth case of DF involing the
Table 1. Review of literature on desmoplastic fibromas of skull
Author Age/Sex Site Tx. Method Recurrence(+/-) Duration of F/U(mointh) Gardini, 1978 7/F Frontal Complete wide resection ? none Hufnagel, 1987 22/F Parietal Complete wide resection ? none
Ovul, 1988 25/M Parietal Complete resection - 24
Okuno, 1990 86/F Temporal Local exicion and curettage - 24
Goldberg, 1994 42/F Frontal Complete wide resection - 72
Selfa-Moreno,1995 28/F Parietal Complete wide resection - 36 Pensak, 1997 21/F Temporal Temporal craniotomy and petrosectomy - 48 Pensak, 1997 28/F Temporal Temporal craniotomy and petrosectomy - 18
Celli, 1997 64/F Parietal Complete resection - 12
Kim, 2001 22/M Parietal Complete wide resection - 35
Fig. 3. Left：Infiltrative growth to the bone showing entrapped sclerotic bony trabeculae(H & E, ×40). Right：Photomicrograph showing neoplastic proliferation of relatively uniform spindle cells in haphazard arrangement(H & E, ×100).
skull. It is suggested that DF of the skull should be best treated by complete resection with a wide margin to prevent a recurrence.
• 논문접수일：2001년 4월 30일
• 심사완료일：2001년 9월 20일
• 책임저자：박 정 율
136-701 서울 성북구 안암동 5가 126-1 고려대학교 의과대학 신경외과학교실 전화：02)920-5712, 5729 전송：02)925-5375, 929-0629 E-mail：[email protected]
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두개골에 발생한 결합조직형성 섬유종；증례보고 및 문헌 검토
고려대학교 의과대학 신경외과학교실,* 적십자 임상센터**
성균관대학교 의과대학 서울삼성병원 병리학교실***
김주한*·박정율*·정용구*·서중근*·김성남**·서연림***
= 국 문 초 록 =
결합조직형성 섬유종은 드문 골종양으로 조직학적으로는 양성이나 국소적으로 빨리 자라는 질환이다. 두개 골에 발생한 결합조직형성 섬유종은 지금까지 9례가 보고되고 있으며, 이 중 8명은 여자에서 발생하였다. 이 논 문은 남자에서 발생한 두개골 결합조직형성 섬유종을 보고하며, 문헌을 검토하여, 이 질환의 임상양상 및 조직 학적소견, 그리고 치료방법등을 보고하고자 한다. 환자는 21세 남자로 두통을 호소하였으며, 두부 전산화 단층 촬영상 뇌을 침습한 소견이 없는 단일 골용해성 부위가 관찰되었으며, 조직학적 검사상 결합조직형성 섬유종으 로 밝혀졌다. 면역조직화학적 염색상, 종양의 에스트로겐과 프로게스테론 수용기는 음성이었다. 정상골을 포함한 종양을 전적출하였으며, 35개월간 추적관찰결과 재발소견은 없었다. 다른 부위의 결합조직형성 섬유종과 비교했 을 때, 종양의 정상골을 포함한 전적출이 두개골에서 가능하므로, 재발이 없는 것으로 생각되며, 남자에서 발생한 결합조직형성 섬유종을 치험하였기에 문헌검토와 함께 보고하는 바이다.
중심 단어：결합조직형성 섬유종・두정골・수술적 가료.
