Celli P, Cervoni L, Trillò G
Department of Neurological Sciences II, La Sapienza University, Rome.
Neurochirurgie. 1997;43(4):260-4.
We present a rare case of desmoplastic fibroma of the skull. Desmoplastic fibroma is a distinctive and rare neoplasm of bone that histologically and biologically mimics desmoid soft tissue tumor. Only 6 cases have been reported in the skull and none of these was diagnosed by MRI.
A 64 year-old woman, operated on in June 1991 for left mastectomy due to a ductal adenocarcinoma and again in October 1994 for a left frontal metastatic adenocarcinoma, was admitted in our Department of Neurosurgery because a control cerebral MRI had detected a diploic lesion, isointense on T1-weighted images and hyperintense on T2, with moderate enhancement, localized in a right parietal site. Neurological examination was negative. The lesion was surgically removed and a cranioplasty was performed. Histological diagnosis was desmoplastic fibroma. Twelve months after treatment she has no neurological symptoms or signs of cerebral lesions (MRI) or systemic metastasis (total body CT).
In the literature the number of desmoplastic fibroma is too small and the follow-up period too short to permit any conclusions regarding the aggressiveness of the tumor.
我们报告一例罕见的颅骨促结缔组织增生性纤维瘤。促结缔组织增生性纤维瘤是一种独特且罕见的骨肿瘤，在组织学和生物学特性上类似于硬纤维瘤样软组织肿瘤。颅骨促结缔组织增生性纤维瘤仅有6例报道，且均未通过磁共振成像（MRI）确诊。
一名64岁女性，1991年6月因导管腺癌接受左乳切除术，1994年10月又因左额叶转移性腺癌接受手术。因脑部MRI检查发现一个板障病变而入住我们神经外科。该病变在T1加权像上呈等信号，在T2加权像上呈高信号，有中度强化，位于右顶叶。神经学检查结果为阴性。该病变通过手术切除，并进行了颅骨成形术。组织学诊断为促结缔组织增生性纤维瘤。治疗12个月后，她没有出现神经症状，也没有脑部病变（MRI）或全身转移（全身CT）的迹象。
文献中促结缔组织增生性纤维瘤的病例数量过少，随访期过短，无法就该肿瘤的侵袭性得出任何结论。
