Objective: The purpose of this study is to explore the clinical characteristics and therapeutic methods of aggressive fibromatosis (AF) in skull. Methods: The clinical data and operative therapy of one case of aggressive fibromatosis in skull in our department were examined and reviewed. The associated literatures were reviewed and discussed. Results: It was indicated that the main manifestation of AF in skull was headache and skull tumor. There was prominent osteolytic destruction found in X-ray plain film for skull AF. And CT scanning showed that skull sclerotin was disorganized and inhomogeneous, with widen diploe. The skull fibromatosis constituted by fibroblasts and myofibroblas, which were mainly spindle-shaped without heteromorphism. Immunohistochemistry showed positive expression of β-catenin and Vim in these cells. The enlarged incision was adopted for the strategy of operation in this patient of skull AF. After follow-up, there was no recurrence of AF discovered. Conclusions: Skull AF is very rare in neurosurgical clinic. The clinical manifestation and iconography of AF were lack of specificity. Therefore, skull AF is hard to diagnose preoperatively. The effective diagnose is mainly dependant on histopathologic examination. As for treatment, operation is the most optimal method so far, which has a good therapeutic effect.
Aggressive fibromatosis (AF), also named as desmoplastic fibroma (DF), desmoplastic fibroma, is a kind of scarce tumor of monoclonal fibroblast/myofibroblast. This disease was firstly proposed and named by Muller in 1838. In 1978, Gardini et al. [
A child patient, fourteen years old, male, was admitted to our department due to a painless masses found in his occiput that was gradually enlarged for 6 months on 10th, February, 2014. Prior to admission, cranium CT of this case in other hospital revealed occipital lesion. This patient did not undergo regular treatment. There was not any cranium trauma history and inheritance history. Medical examination showed that body temperature was 36˚C, pulse was 80/min, with respiration 20/min, blood pressure was 95/43 mmHg. On the skull of inion region, a 4 cm × 4 cm sized hard mass was percepted absence of tendemess and positive sign of Nervous System (NS). Laboratory examination showed the homogram, function of blood coagulation and liver, kidney function were all normal. Moreover, the tumor markers, including carcinoembryonic antigen (CEA), cancer antigen (CA), α-fetoprotein (AFP) and prostate specific antigen (PSA) were all showed normal.
Cranium CT revealed that the diploe of occipital tuberosity was widened with uneven density. Within the diploe of occipital tuberosity, some sand granule-like pykno-shadows were found. Corresponding endosteal and ectosteal lamella were eroded to be thinned with parts of excalation in sclerotin of ectostel lamella. Adjacent soft tissues were not found to beeroded (
Macropathology of lesion skull tissues removed were off-white in color, 5 cm × 5 cm × 0.8 cm sized substantive tumor that was tenacious without envelope. The normal structure of bone tissue was destroyed and it was undis- tinguishable. Microscopy revealed that tumor cells were composed of fusiform cells which were differentiated
well. These fusiform cells distributed asystematically. Parts of them turned out to be braiding and dermatoglyphic pattern. Under the microscope, local hyaline degeneration was found among which were scattered infiltrative bone tissues that were destroyed. The bone tissues exhibited nodule-like in shape, and were encased by fibrous tissues. The nuclei of bone tissues displayed as oval shape. No heteromorphism phenomenon was discovered (see
Cranium AF is a kind of rare tumor. Abroad data revealed that its incidence rate occupied around 0.3% among all benign bone neoplasms, and 0.06% of all bone neoplasms [
been indicated by cellular cytogenetics studies that there exist gene abnormality in the 8th and 20th trisome, the 5th chrosome of APC and β-cateni [
In summary, cranium AF possesses following characteristics: 1) X-ray plain film of skull shows osteolytic destruction in that the lesion could form soft tissue mass, which commonly had a vague boundary to surrounding soft tissues. 2) Plain CT scan reveals that there are some characteristic alterations, including destruction of sclerotin structure, thinningz of endosteal and ectosteal lamella or destruction, widen of diploe, unevenness of bone density. Notably, the enhancement scanning of CT showed reinforcement of focus in common. 3) Lesions in the T1 weighted image of MRI exhibit isointensity and hypointensity, whereas hypointensity in T2 weighted image. There are hyperinsensities found in local areas which are prominently reinforced under enhancement scanning. CT and MRT scan can better reveal the ultra-structure of lesions and sclerotic alterations of endosteal and ectosteal lamella of skull and diploe, which plays a crucial role in ascertaining the scope of tumor boundaries and the invasive extent of surrounding tissues. However, characteristics of iconography above mentioned lack of specificity, various diseases of cranium and meanings have similar iconography manifestation, including CT and MRI [
Therefore, pathological examination is the most dominant method for the diagnosis of cranium AF. The macropathology features of cranium AF are implicated with off-white in appearance, parenchymatous and tenacious in texture, without envelope. Apart from these, the tangent plane of lesion bone tissues is similar to the glu. Moreover, the adjacent skull tissues are always eroded [
Owing to the lack of specificity in clinical manifestation and iconography in cranium AF, it is vulnerable to be error diagnosed as following diseases. 1) Cranium eosinophilic Granuloma. Both diseases have similar iconographic manifestation. But the latter always has low-grade fever. The macropathology of focus in cranium eosinophilic Granuloma exhibits beige or isabeling flexile granulation tissue,while under the microscope, there are a great amount of Langhans’ cells and granulations affluent with blood vessels in the osteolysis destructive area, with a great deal of acidophilic cells. CD1α and S-100 immunohistochemistry can distinguish these two diseases [
So far, operation is the main method to treat cranium AF. Among all the patients cases of cranuum AF had been performed the operation. Due to its local invasive growth style, 16 cases among all cases reported underwent extended extent incision. Following two months to six years’ follow-up, recurrence and transferring were not found in even one case of cranium AF patients investigated. It is indicative that extended extent operation, as the preferred therapeutic mode, has a good prognosis in therapy. In clinical practice, parts of cranium patients may belong to circumstances that were inappropriate to perform operation, or experienced operations without radical excision. Another patients encountered recurrence after operation and were not appropriate for operation again. And some patients did not want to receive operative therapy due to other reasons. The latter may need another adjunctive therapy. However, there are few and available effective adjunctive therapeutic strategies reported until now. Owing to its multivariate biologic behavior, AF has a natural cure to a certain extent. The guidelines of Soft Tissue Neoplasms in synthetic tumor National internet of America figured out in 2012 that observation and waiting also seem to an available therapeutic strategy. Therefore, some cranium AF patients may be given clinical close observation. Limited to fewer cases of cranium AF reported, and the fewer years to be followed-up, the recurrence rate of it has not been statistically studied. So, the future clinical research of cranium AF still need cases accumulation and longer-term follow-up. Taken together, cranium AF is rare in clinic. Due to the non- specific features in its clinical manifestation and iconography, it is hard to pre-operative diagnose for cranium AF. The diagnosis of this disease mainly depends on the histopathilogical examination. The operation is the major therapeutic strategy of cranium of AF. The prognosis of patients who subjected to the operative treatment is relatively good. Feuvret L et al. [
It has been concluded that radiotherapy is not contraindicated for the therapy of this disease. In future clinical research, the radiotherapy for therapy of AF should be applied, and the therapeutic efficiency of it will be intensively studied.
This study was endowed by Yunnan province science and technology project, Grant No. 2015FB008, which is taken charge by Associate Professor Li Jintao.
QiYan,MingLi,JintaoLi, (2015) 1 Case Clinical Report of Cranium Aggressive Fibromatosis and Literature Review. International Journal of Clinical Medicine,06,734-739. doi: 10.4236/ijcm.2015.610098