Oral Manifestations of Neurofibromatosis: A Rare Case Report.

Neurofibroma (NF) is a rare benign non-odontogenic tumor of the oral cavity. NF may present either as solitary lesions or as part of the generalized syndrome of NF or von Recklinghausen’s disease of the skin. The heterogeneous nature of NF was established by Riccardi et al. and he recognized at least seven types of NF. Among these proposed classifications of the disease, two distinct forms are generally accepted namely, a peripheral form known as NF Type I (NF-I), and a central form known as NF-II. NF-I represents the classic form of this disease, described by Recklinghawsen in 1882. Clinically, oral NF usually appears as slow growing, painless, pedunculated or sessile nodules. For illustration, a case of a NF with oral findings is been presented.

Neurofibroma arising from phrenic nerve.

We report a case of neurofibroma arising from the left phrenic nerve and not associated with neurofibromatosis in a 46-year-old male. The patient presented with gradually progressive breathlessness and pain in chest for six months. Radiological investigations revealed a posterior mediastinal mass on the left side of the chest. On postero-lateral thoracotomy, the resected tumour was found to be arising from the phrenic nerve. Histopathological examination confirmed it to be a neurofibroma. Absence of any other distinctive lesions of neurofibromatosis makes this an unusual presentation.

[Solitary neurofibroma of mandible in a 10 years old girl; report of a case]

Neurofibroma is the most common type of peripheral nerve neoplasm. It arises from a mixture of cell types including schwann cells and perineural fibroblasts. It can arise as solitary tumor or may be a component of neurofibromatosis. The skin is the most frequent location for neurofibroma. Lesions of the oral cavity are uncommon, but when occur, the tongue and buccal mucosa are the most common intraoral site. On rare occasions, the tumor can arise centrally within bone, either as a well demarcated or poorly defined unilucular or multilucular radiolucency. In this article, a case of solitary intraosseous neurofibroma of mandible in a 10 years old girl with a discussion on its clinical, radiological, and histopathological characteristics is reported

The Radiological Spectrum of Orbital Pathologies that Involve the Lacrimal Gland and the Lacrimal Fossa

CT and MRI are utilized to differentiate between different types of masses and to determine the extent of lesions involving the lacrimal gland and the fossa. Although many diseases that affect the lacrimal gland and fossa are specifically diagnosed by imaging, it is frequently very difficult to differentiate each specific disease on the basis of image characteristics alone due to intrinsic similarities. In lacrimal gland epithelial tumors, benign pleomorphic adenomas are seen most commonly with a well defined benign appearance, and a malignant adenoid cystic carcinoma is seen with a typical invasive malignant appearance. However, a malignant myoepithelial carcinoma is seen with a benign looking appearance. Lymphomatous lesions of the lacrimal gland include a broad spectrum ranging from reactive hyperplasia to malignant lymphoma. These lesions can be very difficult to differentiate both radiologically and pathologically. Generally, lymphomas tend to occur in older patients. The developmental cystic lesions found in the lacrimal fossa such as dermoid and epidermoid cysts can be diagnosed when the cyst involves the superior temporal quadrant of the orbit and manifests as a non-enhancing cystic mass and, in case of a lipoma, it is diagnosed as a total fatty mass. However, masses of granulocytic sarcoma and xanthogranuloma, as well as vascular masses, such as a hemangiopericytoma, are difficult to diagnose correctly on the basis of preoperative imaging findings alone. A careful clinical evaluation and moreover, a pathologic verification, are needed. In this pictorial review, the various imaging spectrums of pathologic masses involving the lacrimal gland and fossa are presented, along with appropriate anatomy and pathology reviews.

Extreme lateral transcondylar approach to the skull base.

In this study, the authors present their experience of using extreme later transcondylar approach (ELTC) for treating 7 patients with lesions in the anterolateral foramen magnum, upper cervical spine and cerebellopontine angle reaching upto jugular foramen. The tumours included meningiomas, neurofibromas (2 cases each), chondrosarcoma, epidermoid and aneurysmal bone cyst (one case each). The approach was used alone, in combination with retrolabyrinthine presigmoid approach in a patient with lower cranial nerve neurofibroma extending extracranially through the jugular foramen, or in combination with partial C1-C3 laminectomy in two patients with meningiomas situated anterolateral to the cord from the foramen magnum to C3. In two patients with extradural vertebral artery (VA) entrapment by a chondrosarcoma and aneurysmal bone cyst respectively, the vertebral artery was ligated distal to the tumour. The tumours were totally excised in five cases and partially in two. There was no preoperative mortality. The major complications included cerebrospinal fluid leak from the wound (3 cases) and increase in lower cranial nerve paresis (2 cases). At follow up, ranging from 6 months to 2 years, 5 patients showed no tumour recurrence. There was improvement in neurological status. One patient, with a partially excised aneurysmal bone cyst, showed no added deficits or increase in the tumour size. However, there was a massive regrowth in the patient with chondrosarcoma after 6 months. This technique provided a wide surgical exposure with direct visualization of the tumour-anterior cord interface, early proximal control of the VA and preservation of lower cranial nerves.