Glomus jugulare tumours: a 15 year radiotherapy experience in South Australia.

Glomus jugulare tumours (GJT) are one of a family of benign hypervascular neoplasms that arise from chief cells of the paraganglionic tissue from the dome of the jugular bulb. Historically, these tumours have primarily been managed surgically but radiation is an alternative treatment modality. The purpose of this retrospective review was to determine the patient survival, tumour control, clinical control rates and long term toxicity of GJT treated with radiation in South Australia. Between 1996 and 30 June 2012, 15 patients with GJT (16 tumours) were managed with radiotherapy. Twelve patients were female and the median age was 62 years. Thirteen patients (87%) were treated with conventional external beam radiotherapy and two patients (13%) with stereotactic radiosurgery. The mean duration of follow-up was 4 years (range 1 month-15 years 4 months). The close-out date for survival analyses was 31 October 2012. Crude overall survival, tumour control, clinical control and long term grade >2 toxicity rates were 100%, 94% (15/16), 94% and 7% (1/16), respectively. The Kaplan-Meier 5 and 10 year clinical and tumour control rates were both 93% (95% confidence interval: 61-99%). Radiation is an effective treatment modality for GJT. With comparable patient survival, arguably improved tumour and clinical control rates relative to surgery, minimal toxicity and ongoing advances in radiotherapy technologies, radiation should be strongly considered for the primary management of GJT.

Paediatric nasopharyngeal rhabdomyosarcoma: A case series and literature review.

Rhabdomyosarcoma (RMS) is the most common soft tissue tumour in children, with the head and neck region accounting for 35-40% of cases. Nasopharyngeal RMSs tend to grow rapidly and invade adjacent structures. Both the Intergroup Rhabdomyosarcoma Studies and the European Studies have established that the ideal management of this disease is multimodal, using a combination of surgery, chemotherapy and radiotherapy. This case series examines the role of radiotherapy in the management of paediatric nasopharyngeal RMSs, with particular reference to long-term morbidity and disease-free survival. The cases of five children with nasopharyngeal RMS were reviewed and a systematic review of the literature contained in the PubMed databases was conducted to establish 24 individually detailed cases. Management in all patients was multimodal, using a combination of chemotherapy, radiotherapy as well as surgery. External beam radiotherapy is an integral component of treatment for nasopharyngeal RMSs. With more patients surviving for longer periods, more long-term sequelae of radiotherapy have been reported. Complications include sensorineural deafness, endocrine manifestations following radiation of the pituitary gland, cranial nerve palsies, second malignancies within the radiation field, cataract formation, retinopathy and growth disturbance. Morbidity from radiotherapy may be considerable and depends on the field and dose of radiation. Current advances in radiotherapy are aimed at improving the rate of tumour control and reducing such complications. Recent improvements in imaging and conformal techniques have the potential to reduce the morbidity associated with radiotherapy in this cohort.

Stage I seminoma in a 15-year-old boy.

Seminomas are very rare tumours in children and adolescents. We describe a case of seminoma in a 15-year-old boy who was managed with orchidectomy but no adjuvant therapy. He remains relapse-free 8 years after surgery as determined by clinical, radiological and serological surveillance. This study emphasizes the uncertainty over the need for adjuvant treatment in the management of seminoma in the adolescent patient, in particular in prepubescent children.

Childhood glioblastoma multiforme of the spinal cord.

Astrocytoma accounts for more than 50% of all central nervous system tumours diagnosed, with particular prevalence in the 15- to 34-year-old age bracket, rarely arising in younger children. In 1995, a 7-year-old boy presented in Emergency with a 3-day history of severe radicular back pain and associated muscle spasms, exacerbated by lying on his back. Both bone scan and plain X-rays were unremarkable; however, MRI showed a 3-cm space-occupying lesion at the level of T5-T6. The patient proceeded to biopsy and partial excision of the tumour through laminectomy, histology confirming an anaplastic astrocytoma (glioblastoma multiforme), St Anne Mayo grade 4. Treatment consisted of a radical course of radiotherapy alone, delivering a total dose of 44.8 Gy at 1.6 Gy per fraction. The treatment comprised of three phases using two oblique wedged fields on a 6 MV linear accelerator. The patient remains disease free 7 years post treatment, with the only effect noted being a slight kyphoscoliosis at the site of the laminectomy and radiation. This report highlights the efficacy of combined surgery and radiation therapy in the management of spinal cord glioblastoma multiforme in preventing tumour recurrence, with acceptable morbidity. Further evaluation of the treatment efficacy would be difficult because of the scarcity of such cases.

Glomus tumour in a child.

Glomus tumours are uncommon neoplasms. Very rarely do they present in the paediatric age group. We describe a case of bilateral glomus tumours in a 13-year-old girl that were successfully treated with radiotherapy. The patient remains well 8 years after completion of treatment.

Breast-conserving therapy in young women with invasive carcinoma of the breast.

Higher local recurrence rates have been reported in young women with invasive carcinoma of the breast treated with breast-conserving therapy (BCT). However, age itself may not be responsible for this increased risk of recurrence. To investigate this further, a computerized literature search of MEDLINE was performed using data from 1996 to May 2003. The research was limited to female patients with localized, invasive adenocarcinoma of the breast but also included patients of young age with ductal carcinoma in situ. Women of young age with breast cancer, treated with BCT are at an increased risk of recurrence ranging from 7.5 to 35%. However, the data would suggest that the increased risk is secondary to the association of young age with more aggressive tumours and a positive family history of breast cancer. Other factors that may explain the adverse prognosis in women of a young age include associated genetic abnormalities and the lack of mammographic screening programmes for women of young age. Young age is a risk factor for breast recurrence after BCT. However, management decisions should be based on tumour stage, grade and other related prognostic features rather than on young age alone.

Rothmund-Thomson syndrome and tolerance of chemoradiotherapy.

Rothmund-Thomson syndrome (RTS) is a rare disorder with a predisposition for cutaneous and non-cutaneous malignancy. It is speculated that ultraviolet (UV) sensitivity and deficient DNA repair may account for this predisposition and influence the tolerance of chemoradiotherapeutic management. A case is reported of the management of an RTS patient with squamous cell carcinoma of the tongue who demonstrated increased radiosensitivity and tissue intolerance to chemotherapy.

Haemangiopericytoma of the central nervous system.

The records of four patients presenting with a histological diagnosis of haemangiopericytoma of the central nervous system, in Auckland, New Zealand, between 1970 and 1990 were reviewed retrospectively, with the aim of determining the natural history of the disease and response to various treatment modalities. Three out of the four patients reviewed presented with primary cerebral disease and the fourth with a primary spinal cord tumour. All three cerebral primary patients were initially treated with local surgical excision. All three patients received radical radiotherapy following local recurrence. The first two patients remained disease-free locally although one patient developed a solitary liver metastasis 5 years after radiotherapy. The third patient was referred with multiple cerebral metastases and failed to respond to radiotherapy. The patient with the primary lesion in the spinal cord was treated with local excision followed by post-operative radiotherapy and remains disease-free 17 years after treatment. One patient failed to respond to chemotherapy, prescribed to treat a local recurrence adjacent to the previous radiotherapy field. This was successfully excised subsequently. The patient presenting with multiple cerebral metastases was the only patient to die of this disease. Results suggest that local recurrence is avoidable with adequate wide excision of the primary tumour followed by local radical radiotherapy. The role of chemotherapy remains controversial and no conclusion could be drawn regarding the role of palliative radiotherapy from this study. Active treatment and long-term follow-up are necessary because of the relative aggressiveness of this disease and the propensity for late relapses.

Is phenytoin contraindicated in patients receiving cranial irradiation?

Three recent publications have reported the development of erythema multiforme and Stevens-Johnson syndrome in patients receiving cranial irradiation and sodium phenytoin. Some authors have recommended that patients receiving whole brain radiation therapy and who have had seizures should not be prescribed phenytoin but an alternative anti-convulsant. This article reviews the current literature pertaining to the development of this potentially lethal complication in patients receiving whole brain radiation and phenytoin, with reference to the single recorded case of Stevens-Johnson syndrome in a patient receiving cranial irradiation and phenytoin in Auckland, New Zealand. While the clinical picture in the 16 patients reported in the literature and the current case report differed from the classical form of erythema multiforme, a similar pattern of presentation and outcome appeared in all patients reviewed, suggesting that the combination of phenytoin, cranial irradiation and the gradual reduction of concomitant steroids seem to lead to the development of erythema multiforme and/or Stevens-Johnson syndrome. The data presented, although sparse, suggest that phenytoin should not be prescribed in patients receiving cranial irradiation.

A 20-year review of haemangiopericytoma in Auckland, New Zealand.

The records of all patients registered with a histological diagnosis of haemangiopericytoma in Auckland between 1970 and 1990 were reviewed retrospectively, with the aim of determining the natural history of the disease and the response to various treatment modalities. A total of 24 patients were identified, having a median age of 45 years. Twenty-one patients (87.5%) underwent surgery; the remaining three were deemed to be unfit for surgery. Seven patients (29%) were treated with surgery alone; nine (37.5%) received a radical course of radiotherapy and three (12.5%) received palliative radiation therapy for pain relief and/or dyspnoea. Five patients (21%) received chemotherapy during the course of their disease. Eight of the 24 patients (33%) were alive and disease free, 13 (54%) having died and three (13%) being lost to follow-up. Seven patients (29%) died as a result of metastatic disease. Three of the seven (43%) who were treated with surgery alone are known to be alive and disease free. The three patients who had received palliative radiotherapy, died within 2 months of completing the latter treatment. Five of the nine patients (56%) receiving a course of radical radiotherapy are alive and disease free at present. No local recurrence was noted following surgical excision and postoperative radical radiotherapy, whilst eight (67%) of those initially treated by excision alone developed recurrent disease. None of the patients treated with chemotherapy obtained significant palliation. Results suggest that adequate surgical excision followed by postoperative radiotherapy is effective in controlling haemangiopericytoma and that metastatic disease is at present invariably fatal. The role of chemotherapy needs further investigation.

The value of routine computed tomography scanning of the chest in patients presenting with supradiaphragmatic Hodgkin's disease.

Six patients presenting with supradiaphragmatic Hodgkin's disease are presented to demonstrate the potential benefits of chest computed tomography (CT) scanning as a routine staging procedure. These cases show that CT scanning can detect mediastinal and lung involvement not readily detected by other investigations, and that such findings can influence the radiotherapy plan, the need for extended radiotherapy fields or the use of chemotherapy. Following treatment, CT scanning can be useful to assess treatment response and may permit earlier detection of relapse. The use of chest CT scanning as a routine staging procedure in all patients with supradiaphragmatic Hodgkin's disease is advocated.

Malignant lympho-epithelial lesion of the salivary gland: a case report and review of the literature.

Malignant lympho-epithelial lesion of the salivary gland is an undifferentiated squamous cell carcinoma which is associated with a prominent lymphocyte-rich stroma. This study reviews the current literature pertaining to this rare tumour with reference to the single recorded case of malignant lympho-epithelial lesion of the salivary gland presenting in Auckland, New Zealand. This 26 year old Caucasian male developed a rapidly growing lesion in the right parotid gland and multiple ipsilateral cervical lymph nodes. Treatment consisted of surgery and radiotherapy. The patients is currently disease free. One hundred and eight other cases have been reported in the literature, 82 of whom have Mongolian ancestry (Eskimos and southern Chinese) and 15 of whom are Caucasian. More than 50% of these patients (all Mongolian) have elevated titres of serum immunoglobulin A against Epstein-Barr virus capsid antigen. The age at presentation is variable, ranging from 10 to 86 years, with a slight female predominance. Forty-one per cent of patients present with regional lymph node metastases and 20% with distant metastases. Given the rarity of this disease, treatment is controversial. Current data suggest that early radical combined modality treatment using surgery and radiotherapy leads to prolonged survival and cure with 5 year survival rates greater than 50%.

Bone involvement in Hodgkin's disease.

Bone involvement in Hodgkin's disease is uncommon and seldom encountered at initial diagnosis. Seven cases with osseous involvement were identified from a series of 147 patients with Hodgkin's disease treated at Auckland Hospital from 1980 to 1988. Only one patient was found to have bone lesions at the time of initial presentation. Two patients had multiple lesions and 5 had a solitary lesion. Sites of involvement included the spine, pelvis, femur, humerus, ribs, sternum, scapula and base of skull. Six patients had nodular sclerosing histology and one had mixed cellularity disease. All 7 patients were treated with systemic chemotherapy for their advanced disease, and 5 patients needed local radiotherapy to sites of bone involvement. The radiation dosage schedules were individualized, ranging from 30 Gy to 40 Gy, using either a 6 MeV linear accelerator or cobalt machine. At the time of analysis of this study, 4 patients were in complete remission, 2 patients completed chemotherapy with good response and only 1 patient died of disseminated disease. The current review has demonstrated an encouraging response to treatment and good long term control. We believe that combined-modality therapy is effective in the treatment of osseous involvement in Hodgkin's disease.