Abstract
Cranial fasciitis is a fibroblastic lesion found in the cranium of children three weeks to six years of age. It most commonly manifests as a solitary, rapid growing mass on the scalp with frequent involvement of underlying bone and occasional intracranial expansion. Patients with cranial fasciitis may present with a wide range of associated symptoms. Otologic symptoms such as otalgia, otorrhea, hearing loss and middle ear effusion are not frequently encountered. We present a case of cranial fasciitis with intracranial involvement and associated otologic symptoms in a four year old boy with subsequent follow up 14 years later.
1
Introduction
Cranial fasciitis (CF) is a rare non-neoplastic lesion of the cranium. It is a subtype of nodular fasciitis (NF), a reactive proliferative process of fibroblastic cells originating from superficial and deep fascia . Though CF and NF are histologically identical, CF differs from NF as it generally arises from deep fascial layers and the periosteum of cranial bone and is essentially exclusive to children. Typically, CF manifests as a solitary, rapid growing, firm and painless mass on the scalp . We present a case of CF with intracranial extension and associated otologic symptoms in a four year old boy with follow up 14 years from the initial diagnosis. CF cases have been reported in the neurosurgical and dermatological literature; however, to our knowledge only four cases have been reported in the otolaryngology–head and neck surgery literature .
2
Case report
A four year old boy presented to the emergency department with right otalgia and pre-auricular swelling. No local traumatic event was identified. He was diagnosed with a right otitis externa and was discharged home with topical antibiotics. Subsequently, he was referred to the pediatric otolaryngology department for persisting symptoms. Physical examination disclosed a firm painless mass over the right temporal bone measuring 6 × 8 cm causing the right ear to protrude. The right tympanic membrane appeared opaque and bulging. No neurological signs were observed and the remaining physical examination was unremarkable.
A CT scan of the head without contrast disclosed a well-demarcated mass fixed to the right temporalis muscle overlying the inferior portion of the squamous temporal bone. The tissue extended inferiorly to the zygomatic arch and posteriorly to the pinna. The mass extended intracranially in the middle cranial fossa where there was marked bony erosion and adherence to underlying dura matter. Invasion into the right middle ear cavity and aditus ad antrum with opacity in the mastoid air cells were equally noted ( Fig. 1 ).
The patient underwent a right myringotomy and placement of a ventilation tube and a concurrent biopsy of the temporal bone mass. Upon surgical exposure of the right temporalis muscle, the muscle appeared fibrous and leathery with a white coloration. This initial biopsy was inconclusive requiring a second excisional biopsy.
Histological examination revealed muscle infiltrates of spindle-shaped fibroblasts and small lymphocytes with no features of atypia. There were also a number of eosinophils and polymorphonuclear leukocytes distributed in a scattered pattern. There was evidence of mitosis in some large pleomorphic cells, but these were not atypical. A brown substance had accumulated in cellular areas. The infiltrates surrounded individual muscle fibers and fascicules, creating areas of muscle degeneration ( Fig. 2 ).
Immunohistochemical studies found that the lymphocytes stained positive with leukocyte common antigen. The spindle shaped and larger pleomorphic cells stained positive with vimentin. No cellular immunoreactivity was seen for LEU-6, S-100 protein, cytokeratin and muscle actin. These findings were most consistent with a diagnosis of cranial fasciitis.
The lesion presented as a soft, yellow-pink mass, invading both inner and outer tables of the underlying bone. There was also adherence to the dura. The mass originated from the undersurface of the temporalis muscle, infiltrating the muscle and attaching to the scalp. It advanced inferiorly toward the coronoid process pushing down the superior surface of the right parotid gland and extending into the external auditory canal. The mass was completely excised as well as curettage of the eroded bone down to the area of exposed dura ( Fig. 3 ). Cytogenetic investigation of cells from the right temporal mass indicated normal male chromosomes.
The patient was followed yearly for six years, then again fourteen years post-surgery with no recurrence ( Fig. 4 ).
2
Case report
A four year old boy presented to the emergency department with right otalgia and pre-auricular swelling. No local traumatic event was identified. He was diagnosed with a right otitis externa and was discharged home with topical antibiotics. Subsequently, he was referred to the pediatric otolaryngology department for persisting symptoms. Physical examination disclosed a firm painless mass over the right temporal bone measuring 6 × 8 cm causing the right ear to protrude. The right tympanic membrane appeared opaque and bulging. No neurological signs were observed and the remaining physical examination was unremarkable.
A CT scan of the head without contrast disclosed a well-demarcated mass fixed to the right temporalis muscle overlying the inferior portion of the squamous temporal bone. The tissue extended inferiorly to the zygomatic arch and posteriorly to the pinna. The mass extended intracranially in the middle cranial fossa where there was marked bony erosion and adherence to underlying dura matter. Invasion into the right middle ear cavity and aditus ad antrum with opacity in the mastoid air cells were equally noted ( Fig. 1 ).
