Are we over treating Pineal Parenchymal tumour with intermediate differentiation? Assessing the role of localised radiation therapy and literature review.

Pineal Parenchymal tumour with intermediate differentiation (PPTID) is a rare disorder, first classified by World Health Organisation in 2000. There are very few published data available and optimal management is yet to be determined. Management has varied from surgery alone to craniospinal radiotherapy with or without chemotherapy. We present our experience of PPTID treated with radiotherapy alone. We conducted a retrospective review of patients who were diagnosed with PPTID and treated with radiation therapy at our institute from 2010 onwards. Between January 2010 to January 2013, 5 patients of PPTID were treated at our institute. Median age is 44 (range 24-62). All patients had preoperative MRI scan of brain and spine. Imaging did not identify any spinal dissemination. None of the patients underwent a gross total resection, due to the tumour location and technical difficulties. All patients were treated with external beam radiation therapy to primary lesion only with a dose of 54 Gy in 30 fractions after surgery. 4 patients had good partial response and the remaining 1 has stable disease. After 21.4 months of median follow up no disease recurrence was reported. So far there is no evidence of cerebral white matter abnormalities on MRI scan or neurocognitive disorders. Our experience indicated that localised radiation therapy could be an effective treatment strategy for PPTID, considering the long natural course of the disease and the late adverse effects of intensive treatment.

The management and outcome of patients with germ-cell tumours treated in the Edinburgh Cancer Centre between 1988 and 2002.

AIMS: The aim of this retrospective analysis was to review the outcome of patients with germ-cell tumours treated in the Edinburgh Cancer Centre over the past 15 years, and to see whether there had been any changes over three 5-year cohorts. MATERIALS AND METHODS: Patients referred with gonadal and extra-gonadal primary germ-cell tumours, between 1988 and 2002, were identified from the departmental database, and survival by stage and prognostic group was analysed. RESULTS AND CONCLUSIONS: The proportion of patients with stage I seminoma has significantly increased. The good prognosis of patients with early stage disease is confirmed, with the outcome for some groups of patients being better than expected. There is a non-significant trend to improved results over the three 5-year cohorts. The outcome for patients with stage IV seminoma is worse than would be expected, but numbers are small. The poor prognosis of patients with non-seminomatous germ-cell tumours who fall into the International Germ Cell Consensus Classification (IGCCC) poor-prognostic group is confirmed. Failure of patients with metastatic non-seminomatous germ-cell tumours to achieve a complete response to initial therapy is shown to be a poor prognostic indicator.

Testicular sex cord-stromal tumours: the Edinburgh experience 1988-2002, and a review of the literature.

AIMS: Sex cord-stromal tumours of the testis are uncommon tumours, accounting for around 5% of testicular neoplasms. Treatment is primarily surgical, with no adjuvant therapy of proven benefit. We present a single-centre experience over a period of 15 years. MATERIALS AND METHODS: From 1988 to 2002, 18 patients with a diagnosis of sex cord-stromal tumour were referred to our centre. A retrospective analysis of their case notes was made and a pathological review undertaken. RESULTS: Sixteen were Leydig-cell tumours and two were Sertoli cell. For the Leydig-cell tumours, the median age at presentation was 42 years, 50% presented with a testicular mass and 31% with gynaecomastia. Two patients followed a malignant course: one revealing disease dissemination at initial staging, and a second 12 months after potentially curative orchidectomy. Salvage retroperitoneal lymphadenectomy in the latter patient proved unsuccessful. Clinical outcome correlated strongly with the presence of adverse pathological features described previously in the literature. After a median follow-up of 46 months, two patients have developed progressive disease, and two patients have died, one of metastatic Leydig-cell tumour. No patient defined as being of low malignant potential on pathological examination has relapsed outside our review period of 2 years. CONCLUSION: We confirm the overall excellent prognosis for most of the patients with sex cord-stromal tumours of the testis. Compared with most previous reports, pathological features seem to predict with reasonable accuracy the risk of malignant behaviour, and can adequately inform the subsequent review policy.