Cranial Fasciitis Research Articles

Cranial fasciitis in an infant

Cranial fasciitis is a rare reactive, non-tumoral lesion (pseudosarcoma) that occurs mostly in children below 6 years, with a propensity for the cranium. This type of tumor has been reported to arise from the deep fascia, periosteum or from the fibrous layers that cover fontanelles and sutures. The lesion is usually solitary, firm and painless. Grossly, it is usually unencapsulated but well circumscribed. To our knowledge, only 51 cases have been reported in the literature. Herein, we present the clinical, pathological, and radiological findings of a female infant with cranial fasciitis of the skull, and review the literature.

Cranial fasciitis resembling infantile fibrosarcoma differentiated by genetic assay

Cranial fasciitis resembling infantile fibrosarcoma differentiated by genetic assay

Recurrent cranial fibroblastic neoplasm in a male adolescent: Case report and review of the literature

Paediatric fibroblastic tumours are rare neoplasms, of which cranial fasciitis is the most common. We present a case of a male 7-year-old suffering from a cranial tumour preceded by a mild trauma. The tumour recurred despite radical resection within 8 months. Histologically, neither tumour could be classified as any published pathological entity. Both lesions were described as cellular fibroblastic neoplasms; in addition, the recurrent tumour featured a prominent myxoid matrix. In the 12 months following resection of the second tumour, no further disease recurrence has occurred.

Scalp nodules as a presenting sign of fibrodysplasia ossificans progressiva: A register-based study

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive ossification of soft tissues. Clinical diagnosis is important because trauma from lesional biopsies can exacerbate the disease. We sought to evaluate the frequency of scalp nodules as the presenting manifestation of FOP. We describe 3 infants with FOP who presented with multiple neonatal scalp nodules. We reviewed all 43 cases of this disorder in the French FOP registry. Scalp nodules were found in 40% of cases and usually represented the first manifestation of the disease. All 43 patients had characteristic skeletal malformations involving the great toes (n = 43), fingers (n = 12), and vertebrae (n = 3). Other abnormalities were cerebral malformations (n = 1) and alopecia (n = 2). Histopathologic analysis did not contribute to the differential diagnosis and was interpreted as cranial fasciitis in two patients. Our study was retrospective, and the presence or absence of scalp nodules was not always recorded. Neonatal scalp nodules associated with a characteristic malformation of the great toes are a common presentation of FOP. Physicians should be aware that lesional biopsies can exacerbate the disease and must therefore be avoided. A diagnosis of classic FOP can be confirmed by molecular genetic studies.

Exulcerated, nodular tumor on the occiput of a 29-year-old patient

Cranial fasciitis is a rare variation of nodular fasciitis that occurs in the region of the capillitium. We report on a 29-year-old patient who presented with a 2-month history of a tumor progressively increasing in size located on the occiput. Histological examination revealed a tumor, consisting of tightly packed spindle-shaped cells with underlying myxoid stroma, which extended from the dermis to the subcutis. Actin and vimentin were detected by immunohistochemistry. We established a diagnosis of cranial fasciitis and excised the tumor. Especially when a child or young adult presents with a tumor in the skull area, consideration should be given to cranial fasciitis in the differential diagnosis to avoid unnecessary and possibly very invasive treatment approaches.

Cranial Fasciitis Presenting as a Frontonasal Mass

We report the unusual case of a newborn baby with cranial fasciitis of the frontonasal area. Herein, we present the clinical, pathologic, and radiologic findings of a male neonate with cranial fasciitis of the skull. Cranial fasciitis is a benign fibroblastic tumor related to nodular fasciitis, but it has a predilection for the scalp of children. One case has been described in neonates (Neurosurgery. 2001;48:430-435).

Diagnosing cranial fasciitis based on distinguishing radiological features

Primary skull lesions, albeit rare in the pediatric population, have been well described and classified. These lesions are usually benign and commonly present as a painless mass. The most common lesions are epidermoid, dermoid, and Langerhans cell histiocytosis. Cranial fasciitis, encountered less frequently, is usually not considered in this differential diagnosis. Given such few cases reported, it is commonly misdiagnosed preoperatively. The authors retrospectively reviewed data obtained in 4 patients with cranial fasciitis in whom the diagnosis was based on histopathological findings. In 2 patients the onset of the lesion was spontaneous. One patient had a lesion 4 months following a vacuum extraction and subsequent cephalohematoma formation. One patient developed a lesion following a previous craniectomy. Presentation, imaging studies, and histopathological findings were all reviewed and analyzed. All patients presented with a firm nontender mass. Radiological features included a lytic bone lesion with a mildly sclerotic margin, T1 isodensity, T2 heterogeneous hyperdensity, and heterogeneous enhancement. The enhancing portion was not bright on T2-weighted MR images, likely representing the fibrous component; the nonenhancing portion was bright on T2-weighted images, likely representing the myxoid matrix. Histopathological examination revealed proliferating fibroblasts in a myxoid matrix. Cranial fasciitis is a benign, painless but rapidly growing lesion of the skull mainly limited to the pediatric population. It is histologically similar to nodular fasciitis, a fibroblastic proliferation of varying size. These lesions are often related to trauma but can also be insidious or can develop at a prior craniectomy site. The appropriate clinical picture and distinguishing radiographic features may help to differentiate cranial fasciitis from other lesions of the skull allowing for earlier intervention.

A subset of cranial fasciitis is associated with dysregulation of the Wnt/β-catenin pathway

Cranial fasciitis, an unusual fibroproliferative lesion that occurs in the scalp of infants, is considered a posttraumatic reactive process similar to nodular fasciitis. Its pathobiology has not been investigated. Over the last 15 years, we diagnosed cranial fasciitis in six children; in one case, the lesion recurred after 4 years. This lesion and two others showed aberrant, diffuse nuclear reactivity for β-catenin. One of the lesions with aberrant nuclear β-catenin occurred in a child with a history of familial adenomatous polyposis (FAP) and a germline frameshift adenomatous polyposis coli (APC) mutation, c.878delG. The other APC allele in this tumor showed an acquired nonsense mutation, c.4132C → T. Both these mutations lead to translation of a truncated APC protein. The other two cases of cranial fasciitis with aberrant nuclear β-catenin occurred sporadically. One of these showed a point mutation, c.122C → T, in exon 3 of CTNNB1. This mutation causes replacement of threonine with isoleucine at codon 41, leading to loss of a phosphorylation site in the β-catenin protein. The third case with nuclear β-catenin staining was the single one that showed recurrence. This tumor did not show mutations in exon 3 of CTNNB1 or in exons 8/9/16 of APC. The results of this small study indicate a dysregulation of the Wnt/β-catenin pathway in a subset of cranial fasciitis, suggesting that this subset is pathobiologically related to desmoid fibromatoses rather than to nodular fasciitis. Occasional cases of cranial fasciitis may be associated with FAP and serve as an early indicator of this disease, information that would be important in the early diagnosis of FAP in patients without a family history of polyposis.

A case of cranial fasciitis masquerading as acute mastoiditis

We report a case of infection against a background of pre-existing cranial fasciitis. Case report and review of world literature on cranial fasciitis. Cranial fasciitis of childhood is a benign condition and a rare variant of nodular fasciitis. We present the case of a 10-week-old infant with symptoms and signs consistent with a subperiosteal abscess complicating acute mastoiditis. Subsequent findings showed this to be an infection against a background of pre-existing cranial fasciitis. To our knowledge, this is the first such reported case in the literature. Knowledge of the distinctive histopathological features, coupled with an awareness of the condition, are crucial to establishing a definitive diagnosis of cranial fasciitis and, in turn, to instituting appropriate management. The aetiopathogenesis of the condition remains unclear.

A CASE OF CRANIAL FASCIITIS ORIGINATING WITHIN THE DIPLOIC SPACE OF AN ADULT

Abstract OBJECTIVE Cranial fasciitis rarely occurs as a circumscribed lesion in the diploic space without soft tissue involvement. It is even more rare in adults. We discuss the radiographic and pathological differential diagnosis for cranial fasciitis and review the literature regarding clinical presentation and prognosis. CLINICAL PRESENTATION A 27-year-old woman presented with scalp pain and headaches for several months. On physical examination, she had a raised, firm, tender mass above the right ear that was 3 cm in diameter over which there was mild alopecia. Magnetic resonance imaging scans showed a well circumscribed contrast enhancing mass within the diploic space. INTERVENTION Because of the patient's symptoms and the lack of a diagnosis, magnetic resonance imaging-guided surgical resection of the cranial lesion followed by placement of a titanium cranioplasty was recommended. CONCLUSION A nonspecific radiographic appearance warrants biopsy. Complete excision is the optimal treatment choice when the possibility of malignancy cannot be ruled out.

Cranial fasciitis of childhood: a case report and review of literature

Cranial fasciitis is a rare reactive, non-tumoral lesion (pseudosarcoma) that develops in childhood. It is a subset of nodular fasciitis that preferentially involves the scalps of children. This report presents a case of 24-month-old boy with a previous history of head trauma. A firm nodule, showing rapid growth over 1-month period, was seen at the occipital region. Computerized tomography showed the absence of erosion of the skull bones. The mass was completely excised with ample safety margin. Histologically, the growth was composed of mitotically active spindle-shaped cells embedded in a myxoid matrix. The lesional cells were immunoreactive for smooth muscle actin and the diagnosis of cranial fasciitis was established. No evidence of recurrence was seen 18 months after the diagnosis. This report describes the salient features of the case and discusses the relevant literature. It stresses the fact that cranial fasciitis is a rare lesion that must be included in the differential diagnosis of pediatric skull tumors. It also emphasizes that the correct diagnosis of these lesions is needed to avoid unnecessary surgery and radiotherapy.

Cranial Fasciitis in an 8‐Year‐Old Boy: Clinical and Histopathologic Features

Cranial fasciitis is an uncommon benign disorder characterized by a fibroblast-like cell proliferation, observed almost exclusively in children. Clinically, it manifests as a rapidly growing, solitary nodule in the head or neck area. Underlying bone involvement (cranial cortical erosion) is frequently detected. Histopathologic analysis allows differentiation between cranial fasciitis and fibrohistiocytic or even sarcomatous lesions observed in children. Cranial fasciitis is considered to be a reactive, non-neoplastic disorder and is usually cured by a simple excision. An increased awareness of the clinical and histopathologic characteristics of this entity seems important to establish the diagnosis, to adopt an adequate, conservative treatment and to avoid unnecessarily aggressive procedures.

Postoperative cranial fasciitis

The authors report two cases of cranial fasciitis occurring at prior craniotomy sites. They review the presentation and pathological features associated with cranial fasciitis and describe two unusual cases and their treatment. The first case is that of a 16-year-old girl who underwent suboccipital craniectomy for resection of medulloblastoma and 14 months later was found to have a 4-cm nontender mass at the incision site, with evidence of skull erosion on neuroimaging. Resection of the mass revealed cranial fasciitis. The patient later developed two more lesions in the cranial region, as well as lesions on the chest wall and abdomen consistent with nodular fasciitis; all of the lesions were resected. The second case is that of a 61-year-old man who underwent suboccipital craniectomy for hypertensive hemorrhage and 2 years later was found to have an enlarging mass at the incision site, causing compression of the cerebellum. The mass was resected and found to be consistent with cranial fasciitis. Cranial fasciitis is a rare, benign lesion of the cranial region. It is histologically identical to nodular fasciitis, a self-limiting fibroblastic process of the superficial and deep fascia. Although most cases of cranial fasciitis are reported to occur spontaneously in the very young, the two cases reported here involved older patients and lesions that developed at prior craniotomy sites in a delayed fashion, a phenomenon not previously reported. Interestingly, one patient exhibited lesions in other areas as well.

Cranial fasciitis: An unusual ethmoidal mass

Cranial fasciitis is a rare benign tumour involving tissues in proximity to the skull that arises in children, predominantly male. As in the case presented here in a 2.5-year-old boy, it causes symptoms secondary to mass effect. This unusual presentation as an ethmoid mass presented at the medial canthus causing proptosis and epiphora. Whilst referred to sometimes as a pseudosarcoma due to its rapid growth and histological appearance, it is in fact a distinct benign lesion that is successfully treated by excision.

Congenital Cranial Fasciitis—‘‘Watch and Wait’’ or Early Intervention

Cranial fasciitis is a rare variant of nodular fasciitis of childhood, a benign condition cured by surgical excision. Recently, spontaneous involution has been reported and a ''watch and wait'' approach was recommended. We report an instance of a congenital cranial fasciitis that grew rapidly during a 10-month follow-up after fine needle aspiration and incisional biopsy. Conservative surgical excision was curative with no recurrence over a follow-up period of 4 years. Therefore, we recommend that early excision may be necessary in lieu of a ''watch and wait'' approach for large, rapidly growing cranial fasciitis.

Cranial fasciitis with exclusive intracranial extension in an 8-year-old girl

Cranial fasciitis with exclusive intracranial extension in an 8-year-old girl

Fine-needle aspiration cytology and immunocytochemistry of soft-tissue tumors and osteo/chondrosarcomas of the head and neck.

The purpose of the study was to present the clinical and cytological findings of 28 cases of malignant or borderline mesenchymal tumors of the head and neck, of which 22 originated from soft tissue and 6 were found in bone or cartilage. The basic procedures employed involved a cytologic review and subclassification of fine-needle aspiration (FNA) smears from the tumors, which were diagnosed as: pleomorphic sarcomas (5 poorly differentiated, 1 angiosarcoma, and 1 epithelioid sarcoma), spindle-cell sarcomas (2 leiomyosarcomas, 2 malignant mesenchymal tumors, and 1 malignant schwannoma), myxoid sarcomas (2 liposarcomas and 1 high-grade tumor), round-cell tumors (1 malignant histiocytic tumor and 1 chloroma), osteosarcomas (3), chondrosarcomas (3), and borderline tumors (2 pleomorphic lipoma, 1 myxolipoma, 1 cranial fasciitis, and 1 fibromatosis). Histological correlation and problems in subtyping on both cytologic and histological material are discussed. It is concluded that FNA cytology can be used with high accuracy to diagnose musculoskeletal tumors in rare sites such as the head and neck.

Cranial Fasciitis in a Child: A Case Report

Cranial fasciitis is a rare fibroblastic tumor which shows a predilection for the scalp and skull of young children. Histologically, cranial fasciitis is identical to nodular fasciitis which is typically found in the extremities of adults, though is unique in that it may present as an osteolytic lesion of the skull. We report a case of cranial fasciitis in a 20-month-old girl, describing the ultrasonography, CT and MRI findings.

Cranial and extracranial fasciitis of childhood: A clinicopathologic and immunohistochemical study

Fasciitis of various types constitutes a distinctive category of soft tissue lesions whose microscopic features are known for their potential to evoke a pathological diagnosis of one or another type of sarcoma. Nodular fasciitis is the archetype of this group of “fibrous tumors”. Cranial fasciitis is considered a nonneoplastic lesion similar to nodular fasciitis, which is seen almost exclusively in infants and children and has unique clinicopathologic features. The current study documents our experience with fibrous-myofibroblastic proliferations in extracranial sites, mainly in the head and neck region of young children, whose histological features resemble those of cranial fasciitis. These lesions were composed of loosely arranged spindle to stellate cells in a myxoid background. One patient in the study had both cranial and extracranial involvement. Some histological overlap between nodular fasciitis and cranial-extracranial fasciitis was noted, but the latter lesion tended to be more uniform in appearance than nodular fasciitis with its fascicular, spindle cell features and variability in histological patterns within the same lesion, unlike the more consistently uniform myxoid appearance of the cranial and extracranial lesions. Immunohistochemically, the spindle cells of the extracranial lesions and the one case of cranial fasciitis co-expressed vimentin and smooth muscle actin. The extracranial lesions had a predilection for children in the first year of life with all cases occurring at or before 2 years of age, unlike nodular fasciitis, which is rarely seen in the first 4 to 5 years of life. The cranial and extracranial fasciitides should be differentiated from the fibromatoses that tend to locally recur, unlike the nonrecurring behavior of these lesions in common with the other types of fasciitis.

Cranial fasciitis of childhood: an incidental finding of a lytic skull lesion

We describe a case of cranial fasciitis presenting as an incidental finding. A 2-year-old girl had skull radiographs performed after sustaining a minor head injury, which revealed a lytic skull lesion. We describe the salient features of the case and review the current literature of this rare benign inflammatory condition.