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OBJECTIVE: Glioblastoma multiforme (GBM) is the most malignant and most common primary brain tumour worldwide. This study was undertaken to investigate the demographics of this tumour in Jamaica as there is to date no such published data. Data from the recently started Intracranial Tumour Registry (ITR) at the University Hospital of the West Indies was used. METHODS: All cases of GBM entered into the ITR between 2005 and 2012 were gathered. Of these, only patients with pathologically proven diagnoses were entered into the study. Demographic data, including age and gender, were recorded. The distribution of the tumours by anatomic location was also documented. RESULTS: Of the 602 patients entered into the ITR up to that time, 42 were found to have histologically proven GBM with a male to female ratio of 2.2:1. There was an age range of 8-92 years with a mean age of diagnosis of 48 years. The majority of the tumours (66.7%) occurred in the left cerebral hemisphere with the most common lobe being the temporal lobe. Two patients (4.8%) had lesions spanning both hemispheres. CONCLUSIONS: This preliminary study reveals that there is a similar gender distribution of GBM within our population compared with the rest of the world. It, however, revealed that the mean age of diagnosis in our population (48 years) is lower than that quoted in the worldwide literature (53 to 64 years). One possible explanation for this is the possibility that many of our GBMs are actually secondary tumours which are thought to arise from less malignant, undiagnosed precursors. The percentage of GBMs occurring in the paediatric population was similar to the rest of the world.

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In March 2010, the first intracranial tumour registry (ITR) in the English-speaking Caribbean was started at the University Hospital of the West Indies (UHWI). This was deemed necessary as the already established Jamaica Cancer Registry only reports on malignant brain tumours. The ITR will collect data on all prospective intracranial tumours, benign and malignant, which are diagnosed histologically at the UHWI. Retrospective information dating back five years was also collected. Data collected so far reveal that between the years 2006 to 2010, a total of 317 cases were entered into the database. Of these, only 45 cases were considered eligible. The issues surrounding this discrepancy are discussed in this paper along with the many challenges experienced in the establishment of the ITR. From these experiences, the authors have also put forward several recommendations that may be useful to other researchers who wish to implement similar systems.

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Pleomorphic hyalinizing angiectatic tumour (PHAT) is a recently described, rare, low-grade soft tissue neoplasm. The lesion is characterized by clusters of hyalinized and thrombosed ectatic vessels alternating with a variably cellular stroma composed of atypical cells, many with intranuclear pseudoinclusions. Other features are inflammatory cell infiltration, haemosiderin deposits, focal calcificationand minimal to absent mitoses. No metastases have so far been described; however, the local recurrence rate has been found to be high. To date, approximately 60 such cases of PHAT and its precursor, “early PHAT”, have been described in the world literature. We report the first known case of PHAT from this institution which occurred in the left loin of a 77-year old woman. Three years previously, a smaller lesion excised from the same location had been called an ancient schwannoma on histology. This is the most commondifferential diagnosis offered for this entity even though the two differ in immunohistochemical profile. ‘Early PHAT’ was also identified on the periphery of the recurrent lesion.

El tumor pleomórfico hialinizante angioectásico (TPHA) – entidad rara, de reciente descripción – es un neoplasma del tejido blando, de bajo grado. La lesión se caracteriza por la presencia de racimos de vasos ectásicos trombosados e hialinizados, que alternan con un estroma celular variable compuesto de células atípicas, muchas de ellas con pseudoinclusiones intranucleares. Otrascaracterísticas son: la infiltración celular inflamatoria, los depósitos hemosiderínicos, la calcificaciónfocal, y la mitosis mínima o ausente. Hasta el presente no se han descrito metástasis. Sin embargo, se ha hallado que la tasa de recurrencia local es alta. Hasta la fecha, aproximadamente 60 de estos casos de TPHA y su precursor el “TPHA temprano”, han sido descritos en la literatura mundial. Reportamosel primer caso de TPHA conocido de esta institución – una anciana de 77 años de edad, a quién se le presentó en la región lumbar izquierda. Tres años antes, una lesión más pequeña extirpada del mismolugar, hubiera sido llamada un schwannoma antiguo en histología. Este es el diagnóstico diferencial más común ofrecido para esta entidad, aun cuando los dos difieren en cuando a perfilimunohistoquímico. El TPHA temprano fue identificado también en la periferia de la lesión recurrente.

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RESUMEN

Pleomorphic hyalinizing angiectatic tumour (PHAT) is a recently described, rare, low-grade soft tissue neoplasm. The lesion is characterized by clusters of hyalinized and thrombosed ectatic vessels alternating with a variably cellular stroma composed of atypical cells, many with intranuclear pseudoinclusions. Other features are inflammatory cell infiltration, haemosiderin deposits, focal calcification and minimal to absent mitoses. No metastases have so far been described; however the local recurrence rate has been found to be high. To date, approximately 60 such cases of PHAT and its precursor, "early PHAT", have been described in the world literature. We report the first known case of PHAT from this institution which occurred in the left loin of a 77-year old woman. Three years previously, a smaller lesion excised from the same location had been called an ancient schwannoma on histology. This is the most common differential diagnosis offered for this entity even though the two differ in immunohistochemical profile. 'Early PHAT' was also identified on the periphery of the recurrent lesion.

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