Malignant transformation of mature cystic teratoma of the ovary: 30-year experience of a single tertiary care center.

OBJECTIVE: To review the authors' experience with this rare disease and describe their management modality and the outcome. MATERIAL AND METHODS: From January 1983 to December 2013, 13 patients with malignant transformation arising in ovarian MCT were treated at the Division of Gynecologic Oncology in the University of Manitoba. Demographic characteristics, symptoms, signs, stage, mode of therapy, and results of follow-up were reviewed retrospectively. RESULTS: Median age at diagnosis was 53 years (range 25-65). The most common presenting symptom was a palpable mass in nine cases. Squamous cell carcinoma (SCC) was found in 38% (five cases), adenocarcinoma in 15% (two cases), anaplastic carcinoma in 8% (one case), and papillary thyroid carcinoma in 38% (five cases). Eight cases were Stage I, two cases were Stage II, and three cases were Stage III. All patients underwent surgery. Five patients received adjuvant treatment with platinum-based chemotherapy + pelvic radiation. Four patients had recurrent disease (two SCC and two adenocarcinoma). Three patients died of disease after recurrence. The median follow up period of the entire patient population was 60 months, with a three-year overall survival of 76%. CONCLUSION: Malignant transformation of MCT is large ovarian tumors that mainly occur in patients in their fifth and sixth decades of life. They often present as incidental pathologic findings after surgery for MCT. SCC has traditionally been the most common pathology, however in the present series, the authors found that papillary thyroid carcinoma was equally common. Platinum-based chemotherapy with pelvic radiation in early stage (including Stage IA) and locally recurrent dis- ease should be offered. Advanced stages and mucinous adenocarcinoma represent a poorer prognosis despite adjuvant treatment. In patients with papillary thyroid carcinoma, conservative surveillance in early stage and adjuvant total thyroidectomy with radioactive iodine treatment in advanced stage disease appears to be an effective treatment.

Does G gamma/A gamma ratio and Hb F level influence the severity of sickle cell anaemia.

Sickle cell anaemia (SCA) exhibits significant variations in clinical presentation in different populations for which several genetic factors including SCA-associated alpha- and beta-thalassaemias, G-6-PD deficiency and elevated Hb F level have been implicated as possible ameliorating factors. Saudi Arabia is unique in that mild and severe forms of the disease occur at a high frequency. We investigated the G gamma/A gamma ratio and Hb F level and correlated these values with the severity of SCA. The results showed that Hb F level varies significantly in both groups of patients with no evident correlation with the mild clinical manifestations. However, G gamma/A gamma ratio correlated significantly with the disease severity where a high ratio was observed in patients with the mild and a low ratio in patients with the severe disease. The results are evaluated and discussed in the light of correlation studies and regression analysis.

The significance of positive Mantoux reactions in BCG-vaccinated children.

As the interpretation of tuberculin skin tests is controversial in subjects who have received BCG vaccine, we administered Mantoux tests to 2588 randomly selected Saudi children aged 5-13, 1945 of whom had been vaccinated with BCG at birth and 643 were unvaccinated. Only 7.8% of the BCG-vaccinated children were Mantoux positive (greater than or equal to 10 mm induration) at the age of 5 years, which was not significantly different from the unvaccinated children. The tuberculin sensitivity rose more steeply with age in the BCG-vaccinated than the unvaccinated children so that the difference between both groups became statistically significant in those aged 12 and 13 (20% versus 3.9%, 15.5% versus 4.1% respectively). These findings support the previously expressed theoretical postulates that BCG-vaccinated subjects display an increased ability to respond immunologically to encounters with environmental mycobacteria. In communities with low prevalence of environmental mycobacteria, this would result in a slow but persistent rise of skin reactivity to tuberculin which, if given time, will become greater than that of unvaccinated subjects.

Diagnostic role of fibreoptic bronchoscopy in tuberculosis in the presence of typical X-ray pictures and adequate sputum.

Fibreoptic bronchoscopy (FOB) showed that of 82 patients who had 'typical' X-ray pictures of tuberculosis (fibronodular infiltrate in the upper lobe), 40 had active tuberculosis, 30 had inactive tuberculosis and 12 had non-specific fibrosis of undetermined origin. The remaining 16 patients with other specific aetiologies of the upper lobe lesions (e.g. carcinoma) had different X-ray pictures such as a mass or alveolar filling of lobar distribution. In communities with a high prevalence of tuberculosis FOB is therefore unlikely to reveal any specific aetiology (apart from tuberculosis) in immunologically competent patients who have 'typical' X-ray picture of tuberculosis. We document, however, that in sputum-producing patients with active tuberculosis, FOB was the exclusive means of diagnosis in 11/27 (41%), compared with 10/13 (77%) in non-sputum-producers. An immediate diagnosis was made in 22% and 38% of cases respectively.

Isoniazid acetylation phenotyping in Saudi Arabs.

AIMS: The present study is designed to investigate the acetylator status in Saudi Arabs. METHODS: Isoniazid (INH) acetylation phenotyping was studied in 136 Saudi Arabs in Riyadh, Saudi Arabia, using a single plasma sample taken 3 h post-INH oral dose of 200 mg. Metabolic ratio (MR) of plasma acetyl-INH (Ac-INH) to INH was used to determine the acetylation phenotype. RESULTS: The MR had a bimodal distribution with an antimode of 1.0. The frequency distribution of slow acetylators (MR < 1.0) was 94.9% (n = 129). Using Hardy-Weinberg Law, the gene frequency (q) of the recessive allele determining slow acetylator phenotype was found to be 0.97. CONCLUSION: INH phenotyping suggests a high frequency of slow acetylators among Saudi Arabs. There was no association between the MR of plasma Ac-INH/INH and age or gender.

Management of post-tuberculous complex aspergilloma of the lung: role of surgical resection.

Of 14 patients with complex aspergilloma complicating healed tuberculosis, 12 underwent lobectomy or pneumonectomy for recurrent haemoptysis. No deaths occurred, though one patient needed re-exploration for bleeding. There was no postoperative worsening of dyspnoea despite a mean forced vital capacity (FVC) of 60% predicted for the patients undergoing surgery and of 20% predicted for two patients with severe restrictive defects, perhaps owing to the fact that there was little or no function in the resected part of the lung, as shown by preoperative isotope ventilation-perfusion scanning, and that patients were under the age of 50 and generally fit. There has been no recurrence of haemoptysis during follow up, which has been from 12 to 33 months. Surgical resection, provided that cases are carefully selected, offers the best chance of cure with low mortality and morbidity.