Occurrence and distribution of pilomyxoid astrocytoma.

PURPOSE: To know the occurrence and distribution of Pilomyxoid Astrocytomas amongst tumours previously diagnosed histologically as Pilocytic Astrocytoma and to assess the clinical impact of this new entity. METHODS: Retrospective Diagnostic review of all cases histologically diagnosed as WHO Grade I Astrocytoma at a single Neurosurgical unit between 1990 and 2003. RESULTS: Of a total of 91 cases identified, 9 were found to have Pilomyxoid histology. Of these, 8 were children (mean age 3.33 years) and 1 adult. 6 tumours were hypothalamochiasmatic in location. The clinical course of Pilomyxoid tumours was aggressive marked by maturation, multiple recurrences and disease control was rarely achieved with single treatment modality as opposed to typical pilocytics. The overall survival of the pilomyxoid group was not statistically different from the pilocytic tumours. CONCLUSIONS: Encompassing all age-groups and locations, Pilomyxoid Astrocytomas constitute about 10% of all tumours previously diagnosed as Pilocytic Astrocytoma. Nearly two-thirds are hypothalamo-chiasmatic in location. Knowledge of this entity is essential for appropriate aggressive treatment and follow-up.

Metastatic malignant melanoma within meningioma with intratumoral infarct: report of an unusual case and literature review.

We report an unusual case of a patient with a sphenoid wing meningioma that after a few years of static radiological appearance presented with sudden deterioration following rapid growth of tumor with intratumoral infarct. The patient underwent surgery and malignant melanoma deposits within the meningioma were demonstrated on histopathological examination. She had a history of a malignant melanoma (MM) excised from the left forearm 10 months ago with no evidence of recurrence. Although metastasis to meningioma has been widely reported, this is only the second report where the primary tumor is MM. In addition, to the best of our knowledge, intratumoral hypodensity from metastasis is unusual. The tumor-to-tumor phenomenon is discussed and the literature is reviewed.

Twenty year review of histopathological findings in enucleated/eviscerated eyes.

BACKGROUND/AIMS: To evaluate the need for routine histopathological analysis of enucleated/eviscerated eyes and changes in indications for eye removal. METHODS: Retrospective review of all enucleation/evisceration histopathology reports over 20 years. Clinical history was correlated with pathological findings. Two 10 year periods (1984-93, 1994-2003) were compared to detect changes in indications for eye removal. RESULTS: In total, 285 histopathology results were traced from 1984 to 2003; 161 and 124 were evisceration and enucleation specimens, respectively. Glaucoma, malignant melanoma, trauma, and retinal detachment were the most frequent diagnoses 1984-1993. Ocular trauma was the most frequent diagnosis 1994-2003, followed by phthisis bulbi and endophthalmitis. Three cases were diagnosed as metastatic carcinoma; all were suspected preoperatively. A fourth case was a diagnostic surprise: adenocarcinoma found in an eye removed for pain and phthisis. Comparison of two 10 year periods showed a decrease in the number of enucleations/eviscerations, perhaps reflecting a decrease in the number of specimens sent. A preference for eviscerations was evident over the 20 years. CONCLUSION: The number of eyes removed and histologically analysed decreased in the period 1994 to 2003, perhaps because of better treatment options, allowing globe preservation. There was a significant shift in the diagnosis in the two time periods, and a preference for evisceration in both. Only one diagnostic surprise was discovered (0.35%). This study does not support the need to send all globes/contents for histopathological examination. However, because of the one unexpected finding, it is recommended where the examination is incomplete or the history of visual loss is unclear.

A tale of the unexpected: Amyloidoma associated with intracerebral lymphoplasmacytic lymphoma.

Amyloidoma is a rare cause for intracranial space-occupying lesions diagnosed on brain imaging. Histology of excised tissue usually reveals the presence of a discrete, λ-light chain secreting plasmacytoma adjacent to an amyloid mass comprising aggregated monoclonal immunoglobulin light chains. We described a patient with intracerebral amyloidoma associated with a localised lymphoplasmacytic lymphoma and no systemic paraproteinaemia, tumour or amyloid deposits.

Primitive neuroectodermal tumour arising within low grade astrocytoma: transformation, de novo or radiation induced? Report of three cases and review of literature.

The transformation from low grade to aggressive astrocytoma is well known. However, the development of a completely different tumour such as a primitive neuroectodermal tumour (PNET) within a low grade astrocytoma (LGA) is rare. Only two cases have been reported to date. We present three cases and review the literature. One case was identified at presentation. A subsequent review of our histopathology database revealed two further cases. All three patients had histologically proven low-grade astrocytoma and received radiotherapy following biopsy. The tumour location was infratentorial in one and supratentorial in two. The mean age at presentation with initial tumour was 20 years. Two patients underwent partial resection for recurrence, one at five and the other ten years later with histological confirmation of low-grade astrocytoma. At subsequent recurrence eight and thirty years following original presentation and eleven years later for the third patient, further tumour debulking was performed. Histology now revealed high grade PNET. Cytogenetics showed a complex karyotype with multiple chromosomal abnormalities in all three patients. All patients died within 1 year of final surgery. Among the six reported cases of PNET arising following prophylactic radiation therapy to low grade astrocytomas, only two occurred within the original tumour. Whether these cases represent transformation of low-grade astrocytoma, de novo formation of new tumour or radiation induced neoplasm is uncertain.

Cervical spine metastasis from adrenal pheochromocytoma.

Metastatic tumours are the most common type of tumours in the spine of which only 10% occur in the cervical spine. We report a rare presentation of metastatic pheochromocytoma of the cervical spine which was successfully treated operatively. Adequate precautions should be taken during surgery as the tumour is very vascular. Radiotherapy is useful in the treatment of residual tumour.

A rare case of recurrent secretory meningioma with malignant transformation.

A 72-year-old woman previously operated for a sphenoid-ridge meningioma, now presented with double vision. Histology showed a secretory meningioma with an epithelial-appearing, malignant component. Malignant transformation in a secretory meningioma is not known. This is the first report of such an occurrence.