[A cecum carcinoma and liver and peritoneal lesions]. / Een caecumtumor én lever- en peritoneumafwijkingen.

A 37-year-old male presented with acute lower right abdominal pain. A CT-scan showed a cecal mass. During laparoscopic right colectomy, multiple liver lesions and peritoneal deposits were seen. Histology confirmed pT4aN0 cecum carcinoma, but the liver lesions were consistent with sarcoidosis, and the peritoneal deposits were suggestive of benign mesothelioma.

Analysis of local recurrence after breast conservative treatment for invasive breast cancer: a single institution cohort.

In a group of 900 patients treated for carcinoma of the breast, we evaluated patient-, tumour- and treatment-related parameters, predicting the success or failure of conservative treatment for invasive breast cancer. Thirty-one patients developed local recurrences which were detected within 0.1-12.1 years after treatment of the primary tumour (with a median of 6.2 years), providing a risk of 2% at 5 years and 9% at 10 years. The locally recurrent tumours and their original primary tumours of 28 patients could be retrieved from the pathology laboratory archives. These 28 tumours of the recurrence group (RG) were matched with tumours without local recurrence, the non-recurrence group, for age at time of diagnosis, duration of follow-up and T- and N-stage. The tumours were studied for type and grade of invasive tumour including the in situ component and involvement of surgical margins. In addition, the expression of cell-cycle proteins, p53, Ki-67 (MIB-1) and BCL-1, as well as HER-2/ neu oncoprotein, estrogen and progesterone receptor were investigated. We found a mean age at diagnosis for the RG of 50 years, and the mean age at time of diagnosis for the whole group of 900 patients was 56 years (p=0.003). Thirty-nine percent of the RG had a positive surgical margin, which was the case for only 18% in the control group (p=0.09). The presence of the in situ component was also correlated with increased local recurrence (p=0.022). Furthermore, local recurrence was also associated with a significantly increased occurrence of distant metastases (p=0.001). We conclude that breast conservative treatment is safe with a low local recurrence rate.

[Diagnostic image (152). A man with a swelling in the groin. Malignant eccrine poroma]. / Diagnose in beeld (152). Een man met een gezwel in de lies. Maligne eccrien poroom.

A malignant eccrine poroma (porocarcinoma) was excised from the right groin of a 78-year-old man.

[Recurrent otitis media and mastoiditis due to atypical mycobacteria]. / Recidiverende otitis media en mastoïditis door atypische mycobacteriën.

An eleven-year-old girl was operated on due to right-sided chronic otitis media with effusion. After three months, an impressive enlargement of the mucosal lining developed, for which thorough debridement of the middle ear and mastoid was performed. Histological examination revealed a granulomatous inflammation, with negative Ziehl-Neelsen staining. Standard bacteriological cultures revealed no pathogenic micro-organisms. Three weeks later the same clinical picture developed, once again followed by extensive surgical debridement. After a thorough diagnostic work-up an atypical mycobacterium was found, namely Mycobacterium abscessus--formerly named M. chelonei subspecies abscessus. Following appropriate antibiotic therapy the patient was symptom-free. Mycobacterial infections should be part of the differential diagnosis of persistent otorrhoea.

[Less operations required due to perioperative frozen section examination of sentinel nodes in 275 breast cancer patients]. / Minder operaties vereist door peroperatief vriescoupeonderzoek van schildwachtklieren bij 275 patiënten met mammacarcinoom.

OBJECTIVE: To determine the reliability of a peroperative frozen section examinations of sentinel lymph nodes in mammary carcinoma. DESIGN: Retrospective. METHOD: In the Reinier de Graaf Hospital and Diagnostic Centre SSDZ Delft, the Netherlands, the results of frozen section from sentinel lymph node investigations of mammary carcinomas from 1997-2000 were compared with the final pathological results. If axillary dissection had been performed on these patients, the histopathological findings of the dissected lymph nodes were also studied. RESULTS: Frozen sections were made of 287 sentinel lymph nodes from 275 patients. A tumour was found in the sentinel lymph nodes of 64 patients and these patients immediately underwent a complete axillary lymph node dissection. For 31 of these patients a tumour was also found in the other lymph nodes. In 29 of these 31 patients, histological examination had shown extranodal extension. The frozen sections from the sentinel nodes of the remaining 211 patients were considered negative. However, in 13 of these patients, the paraffin sections of the sentinel node nevertheless showed a tumour and the remaining axillary lymph nodes were removed in a second operation. In the last 89 patients studied, the sentinel lymph nodes were cut at four levels and stained immunohistochemically at one level for cytokeratins. Accordingly micrometastases were found in the sentinel lymph nodes of 4 of the 13 patients with (false-)negative frozen sections. False-positive results did not occur. CONCLUSION: The major advantage of the sentinel node method in breast cancer is that for women without metastasis present in the sentinel node, axillary dissection is avoided. By means of a peroperative examination of frozen sections, 83% of the patients with a metastasis in the sentinel lymph node (or about one quarter of all patients) were spared from having a second operation for axillary dissection at a later stage.

Lethal hypophosphatasia, spur type: case report and fetopathological study.

Lethal hypophosphatasia, spur type: case report and fetopathological study: Hypophosphatasia (HP) is characterised by severe undermineralisation of the skeleton owing to deficiency of tissue nonspecific alkaline phosphatase. Clinically a perinatal, infantile, childhood and adult type is distinguished. Clinical signs in the perinatal type of HP show considerable overlap with other skeletal dysplasias such as osteogenesis imperfecta type IIA and type IIC, and achondrogenesis type IA. If present, "spurs" of the limbs are diagnostic for HP. We present a prenatally diagnosed case of HP and discuss the differential diagnosis based on clinical, radiological and pathological findings. Our findings indicate that two types of spurs can be distinguished in hypophosphatasia: midshaft type and joint type.

Prenatal ultrasound diagnosis of rhizomelic chondrodysplasia punctata in a primigravida.

Rhizomelic chondrodysplasia punctata (RCDP) is a sublethal autosomal recessive disorder characterized by skeletal dysplasia, microcephaly, mental retardation, congenital cataracts, joint contractures, skin changes, and failure to thrive. Prenatal ultrasound diagnosis has been reported during the second trimester of pregnancy. Prenatal diagnosis is also possible from the first trimester onwards by demonstration of peroxisomal dysfunction in cultured chorionic villous or amniotic fluid cells. In all cases reported hitherto, the prenatal diagnosis was established after the birth of a previous affected child. In contrast to these studies in pregnant multiparous women at risk for RCDP, we report on the first case of prenatal ultrasound diagnosis of RCDP at 19 weeks' gestation in a primigravida. In addition, a complex cardiac malformation associated with hypoplasia of the thymus (DiGeorge anomaly) is described.

Hereditary renal adysplasia: new observations and hypotheses.

Renal agenesis and dysplasia are frequently regarded by pathologists, even pediatric pathologists, as sporadic malformations. We report six fetal autopsy cases of hereditary renal adysplasia (HRA): two pairs of siblings, one case with paternal unilateral renal agenesis, and one case with an autosomal balanced 6p/19q translocation. The main purpose of this paper is to emphasize that nonsyndromal renal agenesis and dysplasia are pathogenetically related and often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. A subsidiary purpose is to present a case of bilateral multicystic dysplasia with a balanced 6p/19q translocation. This observation further supports the assignment of one of the loci for HRA to chromosome 6p.

Improved metabolic control, clinical periodontal status and subgingival microbiology in insulin-dependent diabetes mellitus. A prospective study.

The effect of improved metabolic control on the clinical periodontal condition and the subgingival microflora of diseased and healthy periodontal pockets in 6 ambulatory insulin-dependent diabetes mellitus (IDDM) patients was prospectively studied. Duplicate measurements with a time-interval of 3 days were made every 4 moths for assessment of the metabolic status, the clinical periodontal condition and the subgingival microflora. During the study, patients maintained personal oral hygiene measures as they usually did before the study. Neither supplementary dental prophylaxis nor oral hygiene measures were applied during the investigation. Long-term metabolic control (HbAlc) improved significantly with intensive conventional insulin treatment. Gingival redness decreased significantly whereas gingival swelling showed a not significant trend to decrease. It is suggested that microvascular changes associated with improved metabolic control in diabetes mellitus may mediate the observed change in gingival redness. No effect could be demonstrated for probing pocket depth, probing attachment level, bleeding on probing and the plaque index. Statistical analysis of the effect of improved metabolic control on the subgingival microflora revealed that only the % of streptococci increased significantly in diseased periodontal pockets. In general, no significant changes were found in either healthy or diseased pockets with regard to the bacterial flora associated with periodontal disease. The results of the present study indicate that improved metabolic control in IDDM patients may have no potential impetus for an improved clinical periodontal condition nor on the subgingival bacterial flora. It is concluded that the periodontal condition in IDDM patients may only ameliorate when local oral hygiene measures are applied.

Relationship between bleeding/plaque ratio, family history of diabetes mellitus and impaired glucose tolerance.

In the present study, the relationship between bleeding/plaque ratio, family history of diabetes mellitus and oral glucose tolerance pattern were investigated in 85 non-diabetic individuals without periodontal breakdown. In this group, no relationship between bleeding/plaque ratio, family history of diabetes mellitus and impaired glucose tolerance was found. The results of the present study suggest that the observed variation in bleeding/plaque ratio cannot be explained by impaired glucose tolerance or family history of diabetes mellitus. It is hypothesized that in subjects with impaired glucose tolerance, the periods of elevated blood glucose values may be too short to introduce tissue changes.

Periodontal condition and microbiology of healthy and diseased periodontal pockets in type 1 diabetes mellitus patients.

On the basis of glycosylated hemoglobin (HbA1c) values, 22 type 1 (insulin-dependent) diabetic adults were grouped into patients with near normal (HbA1c less than or equal to 7.7%) and poor (HbA1c greater than or equal to 9.9%) metabolic control. A total of 44 subgingival sites were examined for Actinobacillus actinomycetemcomitans, black-pigmented Bacteroides species and Capnocytophaga species. No significant difference could be demonstrated between patients in the 2 test groups with regard to periodontal condition. Neither age of diabetic patients nor duration of diabetes mellitus influenced the periodontal parameters. In both test groups, pocket depth of 4 mm or more (greater than or equal to 4 mm) was found to be significantly associated with increased swelling, bleeding after probing and amount of marginal plaque. Proportionally high %s of cultivable A. actinomycetemcomitans (mean 4.3%; range 2.8-5.8%), Bacteroides gingivalis (33.2% and 34.6%) and Bacteroides intermedius (mean 4.2%; range 0.001-13.5%) were isolated from diseased periodontal pockets. In diabetic patients with poor metabolic control, B. intermedius was isolated from diseased periodontal pockets with a mean % of 7.2%, range 0.3-12.5%. Independent of the degree of metabolic control, low %s of Capnocytophaga species were isolated from diseased and healthy periodontal pockets, mean 0.9% (range 0.003-3.9%) and mean 1.4% (range 0.04-4.9%), respectively. It was concluded from this study that metabolic control seems to have no direct effect on the periodontium. Furthermore, the rôle of Capnocytophaga species in the pathogenesis of infectious periodontal disease in type 1 diabetic patients seems to be overestimated.(ABSTRACT TRUNCATED AT 250 WORDS)

An unusual case of black teeth.

Loss of enamel and a deep black stain of the teeth in a 40-year-old diabetic patient are strongly suggested to be caused by the daily consumption of a cheap white wine and, possibly, by the chewing of cayenne. The wine proved to be rather acid, thereby promoting abrasion as a result of gnashing, and to contain a high concentration of tannin. The exact role of the tannins is described.