We reviewed the clinicopathological characteristics of 13 cases of calcifying epithelial odontogenic tumour (CEOT) (Pindborg tumour) diagnosed in the Division on Stomatology, Institute for Medical Research, Kuala Lumpur, over a 29-year period. There were eight female and five male patients. These consisted of eight (61.5 per cent) Malays, three (23.1 per cent) Chinese, one (7.7 per cent) Indian and one (7.7 per cent) Melanau. Their ages at presentation ranged from 19-61 years (mean age, 31.8 years). There were 12 central and one peripheral CEOT. Of these, 76.9 per cent of cases were located in the maxilla, the remaining in the mandible. The commonest clinical diagnosis was a dentigerous cyst (66.7 per cent). Enucleation was the main mode of treatment. Histologically, sheets and strands of polyhedral epithelial cells containing eosinophilic, homogeneous globules with Liesegang rings were observed. One case also showed extensive calcification and clear cell differentiation. Immunohistochemistry revealed a variable keratin staining of the CEOT epithelium, confirming its heterogeneity.
Adenomatoid odontogenic tumours (AOT) are benign, hamartomatous odontogenic lesions that not uncommonly mimic a dentigerous cyst radiographically. Such a case as found involving an unerupted left maxillary canine in a 19-year-old Chinese female is described. The differential diagnosis of some common odontogenic cysts and neoplasms occurring in Malaysians, that may present in a dentigerous relationship to an unerupted tooth is discussed. A brief review of the radiographic literature on AOT is also included.
Destructive thyroiditis commonly occurs during the postpartum period, with a prevalence rate of 5% to 16%, and is mainly due to postpartum autoimmune thyroiditis (PPT) and, very rarely, to subacute thyroiditis. The thyroiditis is similar to Hashimoto's thyroiditis and is generally painless in nature, although cases with painful thyroiditis have been described. We report a case of painful destructive thyroiditis occurring during the postpartum period, which was clinically and biochemically indistinguishable from the variant of painful PPT or subacute thyroiditis. Fine needle aspiration cytology showed multi-nucleated giant cells diagnostic of subacute thyroiditis.
A cross-sectional study of 753 Melanesian children in Vanuatu and 904 Malay children in Malaysia included measurement of refractive error and ocular dimensions. All children were between the ages of 6 and 17 years. The prevalence of myopia in Malay children was 4.3% at 7-8 years and 25.6% at 15-16 years with corresponding figures of 0.8% and 4.3% for Melanesian children. The range of refractive error was greater for Malay children at all ages. Mean refractive error for Malay children showed greater hypermetropia, together with a shorter axial length at 6 years, than Melanesian children, but at 17 years the situation reversed and Malay children had more myopia and longer axial lengths than their Melanesian counterparts.
A case of infantile polycystic kidneys diagnosed prenatally by ultrasound is presented here. This condition was confirmed at post-mortem following delivery of the child. The clinical and pathological features of this inheritable disease is discussed.
A unilateral true ankylosis of the temporomandibular joint of traumatic origin and of 23 years duration is reported. Its pathogenesis and treatment are discussed.
An interesting case is described here in which partial airway obstruction resulted in sleep apnoea and which was relieved by adeno-tonsillectomy. Enlarged tonsils and adenoids causing upper airway obstruction represent one facet of a continuum of hypoventilation - sleep disorders and clinical examination with x-rays will help in determining patients at risk of developing these syndromes.
Serum alpha-fetoprotein (AFP) levels and its expression in liver tissue was studied in 50 cases of histologically confirmed hepatocellular carcinoma (HCC). Serum AFP levels were elevated (greater than 20iu/ml) in 35/50 (70%) of the cases, 28 of whom had levels greater than 500 iu/ml, which is highly suggestive of HCC. These results indicate that serum AFP, by itself, is a relatively insensitive diagnostic test for HCC. Although elevated levels in high risk patients provide a specific clue, a negative result does not exclude the diagnosis of HCC. Expression of AFP by tumour cells paralleled that of serum in the majority of cases. However, tissue AFP was negative in 7 patients who had markedly elevated serum AFP. This observation may be a reflection of preferential excretion of the tumour antigen or differential expression of the antigen by the tumour cells. None of the patients with normal serum AFP demonstrated a reaction for tissue AFP. There was no correlation between AFP production and tumour differentiation.
Frontoethmoidal meningoencephaloceles (FEM) are exceedingly rare in the western hemisphere, Australia, and Europe with an estimated frequency of 1 in 40,000 live births. Among the inhabitants of Thailand, Burma, Malaysia, Indonesia, and parts of the Soviet Union, however, the frequency is as high as 1 in 5000, accounting for 15% of all neural tube defects (NTD). Normal maternal serum alpha-fetoprotein (MSAFP) values usually will be found in these cases since most encephaloceles are closed, skin covered defects. Correct interpretation of the sonographic findings is crucial in establishing a diagnosis as well as giving prognostic and recurrence risk information. To our knowledge, this is the first reported case of prenatally diagnosed FEM. Perinatal management, differential diagnosis for disorders associated with this malformation, and epidemiologic information regarding this rare condition are discussed. It is anticipated that the prenatal sonographic findings may be applied to establish this diagnosis in similarly affected fetuses.
Thirty-one patients with systemic lupus erythematosus had membranous lupus nephropathy (MLN). They were divided into two groups. Group I consisted of 13 patients who had pure MLN but the patients in Group 2 had segmental proliferation in up to 35 per cent of their glomeruli. The rest of the glomeruli had purely membranous change. The patients of Group 2 were no different from the other MLN patients in terms of age, sex and race. The extrarenal disease in both groups was extensive and severe. The renal disease was usually associated with the nephrotic syndrome or oedema but was asymptomatic throughout in one patient. Both renal and extrarenal features responded to treatment initially but relapses were frequent and often severe. Relapses often occurred as treatment was discontinued or medication reduced. Survival at six years in Group I was 62 per cent and in Group 2 was 50 per cent. Only one patient died with renal failure although five patients had impaired renal function at death. The chief causes of death were disease of the central nervous system and infection.
One hundred and fifty patients with urothelial tumours were reviewed. They form the majority of patients with bladder cancer referred to the Institute of Urology over the past three years. From the study it becomes very apparent that the majority of patients are seen late in the course of their disease. The results of treatment of patients with early lesions have been satisfactory but the patients with late invasive lesions have very poor prognosis. A plea is made that one be more aware of this condition and that symptoms of haematuria and urinary tract infections should have a full urological assessment early.
Seventy-seven patients who had PAP smear cytology and colposcopic examination in a 2-year period between 1988 and 1989 were reviewed. Those with findings indicative or suspicious of malignancy were subjected to biopsy. All 50 patients thus biopsied were confirmed to have preclinical cancer on histological examination. Compared against histology, PAP smear cytology gave an accuracy rate of 90% (5 false negatives) and colposcopy gave an accuracy rate of 94% (3 false negatives). These results demonstrate that the two techniques are useful as screening tests for preclinical cervical cancer. The results are improved if they are used complementarily. However, there was poor specificity in the categorisation of cervical cancer by both methods. This was probably due to the subjectiveness of the two procedures. The study also raised the possibility of a higher incidence of preclinical cervical cancer in the Chinese ethnic group.
22 patients with proven hydatidiform molar pregnancy were subjected to whole lung tomography. By this technique, lung metastases were detected in four patients when plain chest radiographs had shown no secondaries. In a fifth patient additional nodules not observed on the plain radiographs were seen. The usefulness of this procedure as an adjunct to existing methods of following up of patients with metastatic trophoblastic disease is discussed.
Despite the high prevalence of oral ulcers little is known about what causes them and how best they can be treated. Recurrent aphthous stomatitis (RAS) is a' chronic inflammatory disease characterized by painful recurring ulcerations of the oral mucosa. RAS can be clinically subdivided into four varieties viz - minor aphthous ulcer, major aphthous ulcer, herpetiform ulcers and Behcet's syndrome. A report on 134 patients with RAS is reported. Minor aphthous ulcer (63%) followed by major aphthous ulcer (29%) were the most frequent. A review of current research on RAS is summarized.
Study site: Department of Stomatology, Institute for Medical Research, Kuala Lumpur, Malaysia
Vocal fold sulcus is a cause of dysphonia which has not been recognized until recently. Awareness of its existence combined with use of laryngostroboscopy would enhance the management of this group of patients. Five such cases were treated initially by voice therapy and subsequently combined with microlaryngeal Teflon injections of the vocal cord. Representative photomicrographs and the end results of treatment are presented. A good voice, subjectively and objectively, was obtained in three patients, with satisfactory improvement in the other two.
In the 10-year period from October 1977 to July 1987, 149 cases of primary IgA nephropathy were histologically confirmed through renal biopsies in the Department of Pathology, University Hospital, Kuala Lumpur. The ages of these patients ranged from 5 to 72 years, with the majority in the 20-30 year age group. There was no sex preponderance. The ethnic distribution showed a significant predominance of Chinese with 107 (71.8%) Chinese, 24 (16.1%) Malays, 15 (10.1%) Indians and 3 others. A wide range of renal glomerular pathology was seen, the commonest being diffuse mesangioproliferative glomerulonephritis (59.1%). Focal proliferative glomerulonephritis (14.1%) followed by minimal change glomerulonephritis (10.7%) were next in order of frequency. Immunofluorescence studies consistently demonstrated heavy and predominant IgA deposition in the mesangium. Weak deposition of C3, IgG and IgM were also observed in various combinations.
In the 7-year period between 1980 and 1987, six cases of childhood primary hepatocellular carcinoma (PHC) were confirmed histologically in our institution. Hepatitis B surface antigen (HBsAg) seropositivity was confirmed in five of the cases, and tissue HBsAg was shown in four of these using the Shikata's orcein stain. An associated maternal HBsAg seropositivity was shown in two of the seropositive children. The youngest seropositive patient who developed PHC was 7 years old. The mother of this patient was also seropositive. These observations support a causal relation between childhood Hepatitis B virus infection and PHC. The importance of vertical or perinatal transmission of HBV in the causation of childhood PHC and the prophylactic role of childhood vaccination is emphasized. Attention is also drawn to the relative short malignant transformation time seen in some of these patients.
Between 1980-1986, 219 renal biopsies were performed on patients with lupus nephritis (LN) presenting at the General Hospital, Kuala Lumpur. There were 172 (78.5%) females and 47 (21.5%) males. The ethnic distribution of 48.4% Malays, 46.1% Chinese and 5.5% Indians reflected their proportional composition in the general population. Peak incidence (40.6%) of cases occurred in the third decade of life (20-29 group) followed by 26.5% and 20.1% in the second and fourth decades respectively. The median age was 24 for females and 27 for males. In both sexes, nephrotic syndrome was the commonest mode of presentation (62.2%) followed by proteinuria (20.5%). Acute oliguric renal failure occurred in 11 patients (5%) and 8 of these showed crescentic glomerulonephritis with more than 50% crescents. The commonest histological picture was diffuse proliferative LN (WHO Stage IV-44.7%) which included 70% (19/27) of those with crescentic disease. This was followed by membranous LN (28.8%) of which 6 (all males) had crescentic disease. 7 (12.3%) of our patients had crescentic nephritis with a female to male distribution of 14: 13, suggesting either more aggressive disease or delayed diagnosis in males.
Key words - Renal biopsies, lupus nephritis, nephrotic syndrome, proteinuria.