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Cranial Fasciitis of Childhood : A Case Report Yoshiaki Kurnon, M.D ., Saburo Sakaki, M.D., Masaharu Sakoh, M .D ., Kei Nakano, M.D., Keiji Fukui, M.D ., and Kenji Kurihara, M .D . Department of Neurosurgery, Ehime University School of Medicine, Ehime, and Department of Neurosurgery and Department of Pathology, Uwajima City Hospital, Uwajima, Japan

Kumon Y, Sakaki S, Sakoh M, Nakano K, Fukui K, Kurihara K. Cranial fasciitis of childhood : a case report. Surg Neural 1992 ;38:68-72 .

Cranial fasciitis of childhood is very rare, only 17 cases having been reported in the literature . We report an additional case of this rare disease . The patient was a 5-yearold boy who complained of left exophthalmos and double vision . Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large epidural mass in the left frontal region that had invaded into the underlying anterior skull base . The tumor showed homogeneous, low density with nonhomogeneous contrast enhancement on the CT scans, and low intensity on the TI-weighted and high intensity on the T2-weighted MRI images . A whitish-pink, elastic, hard tumor was revealed in the epidural space in the left anterior cranial fossa, which was totally excised with curettage of the affected anterior skull base . The origin of the tumor was suspected to be the fibrous connective tissue of the sphenoftontal suture . The histological diagnosis was that of cranial fasciitis . There was no evidence of recurrence 1 year postoperatively. Cranial fasciitis of childhood ; Computed tomography ; Magnetic resonance imaging KEYWORDS :

Cranial fasciitis of childhood was first reported as a specific entity by Lauer and Enzinger [5] in 1980 . It occurs in the cranial bones of young children and histologically resembles nodular fasciitis . This disease is very rare, and only 17 cases have been reported in the literature [1-3,5-9] . We present a case of cranial fasciitis, as well as a review of the literature . Case Report On September 7, 1991, a 5-year-old boy presented to an ophthalmologist of Uwajima City Hospital, Japan, Address reprint requests to, Yoshiaki Kumon, M.D., Department of Neurosurgery, Ehime University School of Medicine, Shigenobu-Cho . Onsen-Gun, Ehime 791-02, Japan . Received December 12, 1991 ; accepted January 9, 1991 . © 1992 by Elsevier Science Publishing Co ., Inc .

with a 10-day history of left exophthalmos and double vision. Since computed tomography (CT) scans revealed an intracranial mass, he was referred to the Department of Neurosurgery on the following day . His double vision seemed to be caused by the left exophthalmos, because external ocular movements were not disturbed on either side . There were no other neurological signs or symptoms, and the results of a hematological examination were normal . A plain skull radiograph revealed an erosion of the left anterior skull base . The CT scans showed a homogeneous low-density mass with contrast enhancement in the left frontal region, and a high-density curved line that raised suspicion of calcification of the dura mater attached to the tumor . These radiological findings suggested that the tumor could be located in the epidural space . The anterior skull base and the inner table of the frontal bone were apparently destroyed (Figure 1) . Magnetic resonance imaging (MRI) showed a low-intensity mass on the T1-weighted images (SE 500/38) and a highintensity mass on the T2-weighted images (SE 2,000/ 110) . Coronal and sagittal MRIs revealed extension of the tumor to the orbit and compression of the intraorbital structures (Figure 2) . A left carotid angiograph showed a marked shift of the anterior and middle cerebral arteries to the right, but there were no vascular abnormalities . Surgery was performed on September 12 via a transcoronal skin incision and a left frontal craniotomy . A whitish-pink, elastic, hard tumor was detected in the left frontal epidural space . The tumor was adherent to the dura mater and was easily dissected from it (Figure 3) . The anterior skull base was severely eroded by the rumor and a small part of the periorbita was exposed . Curettage of the affected anterior skull base and frontotemporal bone was carried out meticulously . Ultimately the entire tumor was removed . The tumor seemed to arise from the anterior skull base . Transection of the surgical specimen showed it was a white tumor that contained a gelatinous area (Figure 4) . Histological examination revealed spindle-shapd fibroblasts in an irregular, whorled pattern (Figure 5), and 0090-3019/92/S5-0I1



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Figure 1 . (Left)

Plain CT trap, showing a low-density epidural tumor . (Center) Enhanced CT scans showing nonhomogeneous contrast enhancement of the tumor. (Right) Plain CT scmu in the bone window level showing erosion of the anterior skull base and the inner table of the fronlotemporal con, exits .

Figure 2 . (Left and center)

7'1-weighted sagittal and corona/ MRls showing a low-intensity tumor and revealing the compression of the intraorbital structure by the tumor . (Right) T2-weighted axial MRI showing a high-intensity tumor .



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Figure 3 . Intraoperative photograph showing a whitish-pink tumor in the left frontal epidural region .

4. Photographs of the excised tumor . (Top) The surface attached to the dura mater was smooth . (Bottom) A transectiou of the tumor was white and had a gelatinous area . Figure

10 fill

15

20

staining of the intercellular matrix with alcian blue was strongly positive, indicating the presence of mucin . Staining was negative for S-100 protein and glial fibrillary acidic protein . Thin-walled vessels were noted, and lymphocytes were barely identifiable . However, there was no evidence of osseous metaplasia or mitosis . The histological diagnosis was that of cranial fasciitis . The postoperative course was uneventful, and the patient was discharged without any residual neurological deficits . There were no signs of recurrence on follow-up CT scans taken 1 year postoperatively .

unluulltlunlltllluulliuliuilnulul11lu11nn1uu Discussion Nodular fasciitis is a benign infiltrating tumor of the superficial and deep fascia, and appears histologically as a benign proliferative lesion of fibroblasts and capillaries with a slight infiltration of chronic inflammatory cells . It occurs frequently in the arms, forearms, hands, legs, and trunk, but rarely in the cranium . It presents as a palpable mass with slight tenderness or pain, and it usually grows rapidly . It has no sex preference and has a peak incidence in the fourth decade [9-11 ] . In 1980 Lauer and Enzinger [51 reported tumor-like fibroblastic lesions occurring in the cranium of children whose clinical and histological features resembled those associated with nodular fasciitis elsewhere . They named these lesions cranial fasciitis of childhood, because of the location of the lesion, histological findings, and age of occurrence . These rare lesions were considered to be a variant of nodular fasciitis .



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Figure 5 . Photograph of tumor speci. The tumor is composed of spindlemen shaped fibroblasts in loose myxoid stroma (hematoxylin and eosin, original magnification x 200) .

Cranial fasciitis is very rare, only 17 cases having been reported in the literature [1-3,5-9] . Histologically the lesion consists of a loose, haphazard proliferation of spindle- to-stellate-shaped fibroblasts set in a myxoid matrix, and it shows focal areas of hemorrhage and infiltration of chronic inflammatory cells [5] . The fibroblasts occasionally form a vague storiform pattern . There are mitotic figures, but not atypical ones . There is reactive bone formation, and mature bony spicules are present in the infiltrated calvaria. In our case spindle-shaped fibroblasts were seen in an irregular, whorled pattern, and the intercellular matrix contained mucin . Thin-walled vessels were noted, and lymphocytes were barely identifiable . There was no evidence of reactive bone formation or mitosis . The histological features in the present case were compatible with cranial fasciitis . As mentioned by Adler and Wong [1], the differential diagnosis can be made only by pathological examination of a tumor specimen, Cranial fasciitis has presented as a scalp tumor in all previously reported cases . In our patient, however, the tumor developed in the epidural space in the frontal region . It is thought that cranial fasciitis can arise from the galea aponeurotica, subaponeurotic loose connective tissue, fascial layer, periosteum, or young fibrous connective tissue of the fontanelles or sutures [5] . In the present case the tumor was located in the anterior skull base, compressing the frontotemporal lobe and the intraorbital structures . Destruction of the bone was more severe in the skull base than in the frontotemporal convexity, and dissection of the tumor from the dura mater

was performed relatively easily, suggesting that the fibrous connective tissue of the sphenofrontal suture could have been the origin of the tumor . The underlying bone was involved in 13 of 17 cases, and this involvement extended up to the dura mater in 6 cases . Extension into the subdural space, however, was not observed in any case . Roentgenograms of the cranium usually showed lyric erosion underlying the soft tissue mass . These changes were seen in 10 of 15 cases in which roentgenograms of the cranium were available for examination . Clinically these lesions presented rapidly growing tumors, and the preoperative duration ranged from 3 weeks to 4 months . In spite of the aggressive clinical manifestations of the tumors, cranial fasciitis is benign . Of the 13 cases in which follow-up information was obtained, 12 did not show any evidence of recurrence, even when the excision of the tumors was incomplete . In only one patient who had two lesions in the right parieto-occipital and left temporal regions, three discrete satellite nodules were noted near the left surgical site 8 months after the excision, although the unoperated right lesion was resolving [1) . It is important for neurosurgeons and pathologists to be aware of this discrepancy between the clinical features and the pathological nature of this particular disease entity . One of the characteristics of this lesion is its preponderant occurrence in children . The ages of the 17 patients ranged from neonatal to 1l years . In 3 patients, the lesions were detected at birth, and 1 I patients were younger than 3 years of age . As shown in Table 1, 12



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Table 1 . Cases Previously Reported in the Literature Case 1

First author (year) Lauer (1980)

2

3 4 5 6 7 8 9 10 I1 12 13

14 15 16 17

Baroho (1980) Pasquier (1984) Ringsted (1985) Adler (1986)

Patterson (1989) Coates (1990) Mollejo (1990)

Age

Sex

Location

21 me

M

Rr temporal

6 yr

F M F M F M M M F M M M

Occipital Rt parietal Rt temporal Frontal Temporoparietal Rt temporal Lt frontoparietal Occipitoparietal Lt frontal Rt parietal Lt parietal Rr parietotemporal (no surgery) Lt temporal (surgery) Rr parietotemporal Unknown Lt forehead Occipital

2 mo 5 yr 3 wk 18 me 4 ma 18 mo 7 mo At birth Afewweeks 6 yr 20 mo

7 yr 3 yr 3 .4 yr

11 yr

M F M F

Follow-up" NR when lost to follow-up after 3 yr NR after 4 mo NR after 1 yr NR after 6 .5 yr NR after 3 .8 yr NR after 6 yr Lost to follow-up Lost to follow-up Lost to follow-up Unknown NR after 1 yr NR after 5 yr Resolving Three discrete nodules after 8 mo NR after 7 yr NR after 1 yr NR after 1 yr NR after 1 yr

' NR, no recurrencee

patients were boys, and 6 patients were girls, giving a 2 : 1 predominance of males to females [5} . These lesions occurred frequently in the temporal or parietal regions . There were 4 lesions in the temporal, 3 in the temporoparietal, 3 in the parietal, 2 in the occipital, 1 in the frontoparietal, and 1 in the occipitoparietal region . One patient had two lesions in the right parietotemporal and left temporal regions [1) . The differential diagnosis must be performed by histological examinations for eosinophilic granuloma, inflammatory lesions (such as syphilis or sarcoidosis), fibrous dysplasia, and meningioma [5,8,9] . Infantile fibrosarcoma is more cellular and has a more fascicular pattern than that of cranial fasciitis . In addition it recurs and metastasizes more frequently [3,5,9] . Juvenile aponeurotic fibroma and chondromyxoid fibroma occur in slightly older children and grow more slowly [9] . The occipital mesenchymal tumors of infancy reported by Hockley et al [4] were found to be different from our case in their histological features in spite of being the same intracranial lesions .

References 1.

Adler R, Wong CA . Cranial fasciitis simulating histiocytosis . J Pediatr 1986 ;109(1) :85-88 . 2 . Barohn RJ, Kasdon DL . Cranial fasciitis : nodular fasciitis of the head . Surg Neurol 1980 ;13 :283-5 . 3 . Coates DB, Faught P, Sadove AM . Cranial fasciitis of childhood . Plasr Reconstr Surg 1990 ;85(4) :602-5 . 4 . Hockley AD, Hoffman HJ, Hendrick EB . Occipital mesenchymal tumors of infancy. J Neurosurg 1977 ;46 :239-44 . 5 . Lauer DH, Enzinger FM . Cranial fasciitis of childhood. Cancer 1980 145 :401-6. 6 . Mollejo M, Millan JM, Ballestin C, Serrano C . Cranial fasciitis of childhood with reactive periostitis . Sung Neurol 1990 ;33 :146-9 . 7 . Pasquier B . Keddart P, Pasquier D, Barge M, Bost M, Couderc P . Fasciire cranienne de ['enfant . Ann Parhol 1984 ;4 :371-5 . 8 . Patterson JW, Moran SL, Konerding H . Cranial fasciitis . Arch Dermatol 1989 ;125 :674-8. 9 . Ringsted J, Ladefoged C, Bjerre P . Cranial fasciitis of childhood . Acta Neuropthol(Berl) 1985 ;66 :337-9 . 10 . Soule EH . Proliferative (nodular) fasciitis . Arch Parhol 1962 :73 :1 7-24. 11 . Stour AP . Pseudosarcomatous fasciitis in children . Cancer 1961 :14 :1216-22 .

Cranial fasciitis of childhood: a case report.

Cranial fasciitis of childhood is very rare, only 17 cases having been reported in the literature. We report an additional case of this rare disease. ...
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