Proton Therapy in Head and Neck Cancer Treatment: State of the Problem and Development Prospects (Review).

Proton therapy (PT) due to dosimetric characteristics (Bragg peak formation, sharp dose slowdown) is currently one of the most high-tech techniques of radiation therapy exceeding the standards of photon methods. In recent decades, PT has traditionally been used, primarily, for head and neck cancers (HNC) including skull base tumors. Regardless of the fact that recently PT application area has significantly expanded, HNC still remain a leading indication for proton radiation since PT's physic-dosimetric and radiobiological advantages enable to achieve the best treatment results in these tumors. The present review is devoted to PT usage in HNC treatment in the world and Russian medicine, the prospects for further technique development, the assessment of PT's radiobiological features, a physical and dosimetric comparison of protons photons distribution. The paper shows PT's capabilities in the treatment of skull base tumors, HNC (nasal cavity, paranasal sinuses, nasopharynx, oropharynx, and laryngopharynx, etc.), eye tumors, sialomas. The authors analyze the studies on repeated radiation and provide recent experimental data on favorable profile of proton radiation compared to the conventional radiation therapy. The review enables to conclude that currently PT is a dynamic radiation technique opening up new opportunities for improving therapy of oncology patients, especially those with HNC.

Skull base trauma: Masking, stimulating or neutral factor for the insidious development of angiosarcoma in a 19-year-old.

Angiosarcoma is a malignancy of endothelial tumor and represents 1-2% of all soft tissue sarcomas, uncommonly found in the head and neck region. The etiology is not clear but there are definite risk factors including chronic lymphoedema, history of radiation, environmental carcinogens and certain familial syndromes. Presented here is a case of a patient treated due to the skull base trauma and diagnosed with this type of tumor.

Anatomical variations of clivus: a descriptive anatomical study.

PURPOSE: The clivus is a part of the sphenoid bone. It may show some anatomical variations such as fossa navicularis magna (FNM), canalis basilaris medianus (CBM) and craniopharyngeal canal (CPC). These variations have been associated with conditions like meningitis and tumors of skull base. Inadequate information about these structures may pose a risk of inaccurate diagnosis resulting in unwarranted interventional procedures. Hence, the knowledge about the prevalence of these variations is important. Thus, the objective of this study was to determine the prevalence of fossa navicularis magna, craniopharyngeal canal and canalis basilaris medianus and its types utilizing cone-beam computed tomography (CBCT) images. METHODS: Retrospectively, a total of 350 CBCT scans were evaluated for the presence of FNM, CBM, its types and CPC. The analysis was done by two observers independently. Cohen's kappa statistics was used to determine the interobserver agreement. RESULTS: FNM was noted in 19.4% cases, CBM in 9.7% cases, whereas CPC was not identified in any case. Type 5 was the most common type of CBM. There was no significant difference (p > 0.05) between genders and age groups for the prevalence and a highly significant (p < 0.01) substantial agreement between observers for the prevalence of FNM and highly significant (p < 0.01) moderate agreement for the prevalence of CBM and its types was obtained. CONCLUSION: FNM, CBM, CPC, albeit rare anatomical variations of clivus, knowledge of these structures is important for radiologists, anatomists and surgeons to avoid misdiagnosis and provide awareness to the individual of a higher possibility of meningitis or tumors of the skull base.

Radiation-induced intracranial osteosarcoma of the anterior skull base after treatment of esthesioneuroblastoma.

Esthesioneuroblastoma (ENB) is an uncommon sinonasal cancer of the olfactory neuroepithelium that is typically treated with surgical resection followed by radiation therapy. Radiation-induced intracranial osteosarcoma of the skull base is a rare but devastating long-term complication of radiation therapy in this region. Here, we present a case of an 82-year-old patient who developed radiation-induced osteosarcoma of the anterior skull base and paranasal sinuses 10 years after radiation therapy following resection of an ENB. Older patients may be at risk of developing this complication earlier and with a worse prognosis relative to younger patients. Treating physicians/surgeons should be aware of this devastating complication. Patients who are treated with high-dose radiation therapy in this region should be followed for many years.

Osteoradionecrosis of the skull base.

Radiation therapy (RT) is often necessary for the treatment of head and neck cancers. Osteoradionecrosis (ORN) is a rare, but potentially serious complication of RT. RT leads to the destruction of vasculature in radiated tissue causing hypoxia and tissue necrosis. ORN can occur in any bone, but bones with naturally poor blood supply appear to be more susceptible. Bones of the skull base are susceptible, with ORN occurring in the anterior, central, and lateral skull base. Risk factors include cancer type and location, radiation dose, and a variety of patient factors. Patients often present with pain, bleeding, and foul odor and are typically found to have exposed and necrotic bone. Treatment options vary depending on the severity, but typically include pentoxifylline and vitamin E as well as surgical debridement, with less evidence supporting hyperbaric oxygen therapy. Recognition and prompt treatment of ORN will allow for improved patient outcomes.

Metastatic Pleomorphic Adenoma in the infratemporal fossa and neck following total parotidectomy after 30 years.

Metastasising pleomorphic adenoma is rare and may occur years after surgical excision of a pleomorphic adenoma (PA). We present a 61-year-old woman with a right infratemporal PA with metastases to the cervical lymph nodes after 30 years following a total parotidectomy. She was treated successfully with a resection of the tumour with combined neck and mandibulotomy approach along with postoperative radiotherapy given subsequently.

Skull Base Chondrosarcoma Caused by Ollier Disease: A Case Report and Literature Review.

BACKGROUND: Ollier disease (OD) is a rare, nonhereditary bone disease that is characterized by the presence of multiple enchondromatosis (3 or more) with a typical asymmetric distribution which is mainly confined to the appendicular skeleton. OD's most serious complication is the transformation of an enchondroma into chondrosarcoma. The most common sites for chondrosarcoma are in the pelvic and shoulder bones, the superior metaphyseal and diaphyseal regions of the extremities. However, the cranium is an extremely rare site for chondrosarcoma because of OD. CASE DESCRIPTION: We report the case of a 27-year-old woman who was admitted to our hospital with paroxysmal headaches over 1 month and left ptosis for 2 weeks. Magnetic resonance imaging (MRI) scan revealed a mass was located at the left side of the parasellar area. The mass was surgically removed, and histopathologic examination confirmed chondrosarcoma grade I. During follow-up, more imaging examinations and pathologic examination confirmed the final diagnosis was OD. CONCLUSIONS: Intracranial chondrosarcoma caused by OD is extremely rare but should be considered in the differential diagnosis when primary chondrosarcoma is diagnosed. Preoperative diagnosis is challenging, and definitive diagnosis requires immunohistochemical examination and systematic examination of the body. Surgical resection is the most effective therapy for rapid relief of symptoms. For patients with OD with normal intracranial MRI, long-term follow-up is necessary.

Brain and skull base MRI findings in patients with Ollier-Maffucci disease: A series of 12 patient-cases.

OBJECTIVES: To estimate the prevalence rate of silent cranial and intracranial lesions in a series of Ollier-Maffucci patients. PATIENTS AND METHODS: Cerebral MRI was routinely performed in Ollier-Maffucci patients followed-up in our tertiary centers. Patients with previous history of skull base or intracranial tumors were excluded from the study. Clinical and radiological datas were retrospectively collected. The occurrence rate and nature of abnormal cerebral MRIs were determined. RESULTS: Twelve patients were included. A glioma-looking lesion was found in one patient (8%), while skull base lesions were evidenced in 3 others (25%). A regular MRI follow-up was recommended for each patient, with a time interval varying between 1year and 3years depending on the likelihood of tumoral evolutivity, as infered from the MRI findings. CONCLUSION: All in all, the high rate of intracranial and skull base lesions with a malignant potential warrants to include cerebral MRI in the routine follow-up of Ollier-Maffucci patients.

IMAGING DIAGNOSIS-MAGNETIC RESONANCE IMAGING AND HISTOPATHOLOGICAL FEATURES OF A SKULL BASE CHORDOMA IN A CAT.

An 8-year-old domestic short-haired cat was presented with anorexia, lethargy, ataxia and one episode of consciousness loss. A midline vertically orientated, biconcave, extra-axial mass originating from the basioccipital bone was detected on magnetic resonance images of the head. The mass was T1W iso- to hypointense when compared with normal grey matter, T2W hyperintense with small areas of isointensity and heterogeneously enhanced with contrast. Multiple signal voids were observed on T2* images. Histopathological evaluation confirmed a chordoma. To the authors' knowledge this is the first report of the imaging characteristics of a chordoma affecting the skull base in a cat.

[Etiology and pathogenesis of glomus tumours (chemodectomas) of the base of the skull].

The objective of the present work was to describe the poorly known history of the development of the nomenclature of glomus tumours (chemodectomas) of the base of the skull, elucidate etiology and pathogenesis of these neoplasms. The authors present a chronological table illustrating the progress of etiological and pathogenetic studies as well as the surgical treatment of the tumours. The results of analysis and discussion of the controversial issues of the nomenclature are reported.

Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and Literature Review.

BACKGROUND: Maffucci syndrome is a nonhereditary disorder in which patients develop multiple enchondromas and cutaneous, visceral, or soft tissue hemangiomas. The potential malignant progression of enchondroma into a secondary chondrosarcoma is a well-known fact. Nevertheless, chondrosarcoma located at the skull base in patients with Maffuci syndrome is a very rare condition, with only 18 cases reported in the literature. CASE DESCRIPTION: We report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review. CONCLUSIONS: Skull base chondrosarcoma associated with Maffucci syndrome is a rare condition. The disease cannot be cured, therefore surgical treatment should be performed in symptomatic patients aiming for maximal tumor resection with function preservation. The endoscopic endonasal approach is a safe and reliable alternative for the management of these tumors.

Ectopic prolactinoma in the clivus: a case report.

We report the case of an ectopic pituitary adenoma in a 65-year-old man with an empty sella who initially presented with right ptosis and eyelid edema and headache. Neuroimaging studies revealed a large tumoral process at the height of the clivus, with partial destruction of surrounding bone structure. He underwent transphenoidal surgery and histopathologic examination, including immunohistochemical studies, revealed a prolactin-producing pituitary adenoma. A careful review of the literature was done.

Radiation-induced osteosarcomas of the calvarium and skull base.

BACKGROUND: Although a rare complication of ionizing radiation, radiation-induced osteosarcoma is now more frequently recognized as radiation therapy has become common and cancer survival has increased. To date, publications on radiation-induced osteosarcoma of the cranium are limited to a few small series and case reports. METHODS: Data from 175 patients with a history of sarcoma of the head at The University of Texas M. D. Anderson Cancer Center from 1975 to 2007 were reviewed to identify patients with radiation-induced osteosarcoma. The diagnostic criteria were: 1) osteosarcoma arose within the previously irradiated field; 2) new sarcoma was histologically distinct from the original neoplasm; 3) no evidence of new sarcoma at the time of radiation; and 4) distinct latency period could be recognized. Frequencies and descriptive statistics were obtained for the various characteristics under study. RESULTS: The authors identified 16 patients with radiation-induced osteosarcoma of the cranium at their institution. The average age at diagnosis was 35 years. The median latency period was 12.5 years. Nine patients had skull base tumors, and 7 had calvarial tumors. Of the 14 patients treated surgically, 86% developed local recurrence. The median survival time was 29 months, and the 5-year survival rate was 29.6%. CONCLUSIONS: The authors report the largest series of cranial radiation-induced osteosarcoma. Although radiation-induced osteosarcoma is an uncommon but dire complication of radiotherapy, its incidence will probably increase in the future as the frequency of radiation treatment and cancer survival increase. These tumors are locally aggressive, and despite aggressive surgical and medical management, they have a high rate of local recurrence and mortality.

Conservative management of endolymphatic sac tumors in von Hippel-Lindau disease: case report.

In endolymphatic sac tumors associated with von Hippel-Lindau disease, early detection and surgery have been warranted to avoid associated neurological morbidity. However, in lately discovered tumors, hearing preserving surgery is often impossible and timing of surgical resection is difficult to define. We report two cases of tumors revealed by a sudden and profound hearing loss and managed conservatively for more than 15 years without worsening of the neurological symptoms associated with the endolymphatic sac tumor. Tumor size remained stable for the first patient and a stuttering growth pattern was observed for the second patient. Initial observation may be considered a not unreasonable management paradigm in these cases.

Sarcoma of the sella after radiotherapy for pituitary adenoma.

Secondary malignancies are infrequent sequelae of pituitary radiotherapy. The goal of the present case study is to analyze clinical features of a selected group of cases to define the special characteristics of these tumors. We report the illustrative case of a 38-year-old man with acromegaly who had transsphenoidal surgery and radiotherapy 7 years before presenting with a sellar high-grade sarcoma. Transsphenoidal and transcranial resection, as well as repeated gamma knife radiosurgery, could not prevent tumor progression and development of meningiosis sarcomatosa. We performed a thorough search of the literature and reviewed numerous publications and reports on primary and secondary sarcomas of the sella. Our search revealed 51 cases of mesenchymal malignancies after sellar radiotherapy. For further analysis, we identified and selected a group of patients based on the criteria for studying radiation-induced tumors as described by Cahan.Compared to the surgically treated group, secondary sarcomas of the sella are more frequent in patients who have had radiotherapy. These tumors occur at normal dose schedules with long latencies. Their growth is very aggressive and they may develop meningiosis sarcomatosa. Until now, no treatment modalities have been able to stop the progression of these neoplasms. Radiation-induced sarcoma is a rare sequela of pituitary radiotherapy. It is important for the treating physician to keep in mind the possibility of post-radiation sarcoma development. Additionally, one must include these tumors into the differential diagnosis in pituitary patients presenting with tumor recurrence more than 5 years after radiotherapy in combination with a secondary lack of hormonal activity.

Malignant giant cell tumor in the posterior fossa of a neonate.

Giant cell tumors (GCTs) of the bone are rare, usually benign but locally aggressive neoplasms that primarily occur in the epiphyses of long bones. They seldom develop in the cranium; when they do, they involve principally the sphenoid and temporal bones. These tumors usually affect young adults, and few reports in children have been published. Primary malignant GCTs of the skull are even more uncommon. The 3 published cases all involved adults over 40 years of age. Herein, the authors present a case of a highly aggressive primary malignant GCT of the posterior fossa in a 5-week old preterm infant. One month after the gross-total resection of the tumor found in the bone, the infant's condition rapidly deteriorated and she died. Magnetic resonance imaging and postmortem examination revealed a tumor larger than it had been before the operation, with expansion toward the brain. To the best of the authors' knowledge, this is the youngest patient reported with a primary malignant GCT of the skull, and actually the first case in a pediatric patient. In addition, the extremely high growth rate of the tumor in the postoperative period renders this case the most aggressive primary malignant GCT of the cranium described so far.