A giant guteal schwannoma with extension into the pelvis: a case report.

BACKGROUND: Schwannomas are uncommon slow growing tumours arising from the nerve sheath or Schwann cell. OBJECTIVE: To report a case and the surgical removal of a giant complex schwannoma and to highlight the value of extensive investigations including a preoperative histologic diagnosis in the successful surgical management of uncommon large benign tumours. METHODS: A 39-year-old man presented with a 15-year history of a large mass in the right gluteal region. He was clinically evaluated, subjected to imaging studies and surgery. RESULTS: Clinical examination revealed a 40 cm by 60 cm mass in the right gluteal region and continuous with a 25 cm by 15 cm pelvic mass. The ultrasound scan revealed a very complex (mixed solid and fluid containing) gluteal mass with extension to the pelvis. The CT scan showed a very large, well defined lobulated tumour with cystic spaces and enhancing nodules. The tumour extended through the ischiadic foramen into the pelvis and posteriorly into the thigh muscles. A Doppler scan of the pelvic vessels revealed that the right common and internal iliac arteries were both compressed but not occluded. An incisional biopsy was reported as a Schwannoma with xanthomatous changes and an immuno histochemistry profile of S-100+, Ki-67+ (less than 1% of the cells). A right foot drop following the surgery responded to physiotherapy. The duration of total hospital stay was 12 weeks. CONCLUSION: The staged excision of large and complex schwannomas is safe. It is essential that a preoperative histological diagnosis is made to establish that the tumor is truly benign.

Breast cancer in men: aspects of familial aggregation.

Familial aggregation of breast cancer in males was investigated in a population-based case-control study. Cases were ascertained from 10 Surveillance, Epidemiology, and End Results Program registries in the United States between 1983 and 1986. Controls were identified by random-digit dialing and from lists of Medicare recipients. The relative odds of developing breast cancer were similar in men with affected paternal and maternal relatives and in men with affected mothers and sisters. The risk increased with the number of affected relatives. The relative odds of developing breast cancer were greater in men with first-degree relatives who developed their mammary neoplasm before the age of 45 than in men with older first-degree affected relatives; the enhancement of risk in men with an affected sister was greater in those under age 60 than in older men. These results are similar to those observed by others in studies of breast cancer in women.

Significant expression of IGFBP2 in breast cancer compared with benign lesions.

BACKGROUND/AIM: Insulin-like growth factors (IGFs) and IGF binding proteins (IGFBPs) play a role in the normal development of breast tissue, and possibly in breast cancer aetiology. IGFBP2, one of six members of the IGFBP superfamily, acts as regulator of the IGFs and has pleiotropic effects in normal and neoplastic tissues. Because IGFs have mitogenic effects on mammary epithelia, this study investigated IGFBP2 expression in mammary tissues of different benign and malignant entities. METHODS: Immunohistochemistry was used to study correlations between the presence and intensity of IGFBP2 staining and tumour type and grade, in addition to steroid hormone receptor status, in 120 breast specimens. Expression was measured by quantitative colour video image analysis and semiquantitative evaluation, and the measurements correlated well (r = 0.92; p<0.05). RESULTS: Both methods found no significant expression of IGFBP2 in normal glandular cells and hyperplasia (group I). Atypical hyperplasia showed a slightly increased cytoplasmic expression of IGFBP2, and carcinoma in situ showed a distinctive, membrane associated and cytoplasmic expression (group II). Infiltrating carcinomas strongly expressed cytoplasmic IGFBP2 (group III). There were significant differences between group I and II, and between group II and III. There were no significant differences between invasive lobular and invasive ductal carcinoma, or between grades I, II, and III within these entities. There was no significant correlation between IGFBP2 immunostaining and oestrogen or progesterone receptor positivity within the malignant group. CONCLUSIONS: IGFBP2 mitogenic signals of autocrine/paracrine regulatory mechanisms may be responsible for the growth of breast carcinomas and IGFBP2 may be an independent indicator of malignancy.

Reproductive history and cigarette smoking as risk factors for thyroid cancer in women: a population-based case-control study.

A population-based case-control study was conducted in Northern Norway and Central Sweden to investigate hormonal and reproductive factors and cigarette smoking as determinants of papillary and follicular thyroid carcinoma in women. Information on 191 histologically confirmed cases and 341 age-matched controls was included. No clear association was found with regard to the number of live births, number of pregnancies, a history of incomplete pregnancies, or the use of oral contraceptives or hormonal replacement therapy. However, an early first childbirth (before 20 years of age, or less than 5 years after menarche) was associated with an increased risk of thyroid cancer. There was an increased risk of thyroid cancer among women with a history of artificial menopause compared to those with a spontaneous menopause [odds ratio (OR), 2.52; 95% confidence interval (CI), 0.96-6.62], which was more pronounced for the papillary carcinoma and after adjustment for age at menopause and use of replacement therapy. Cigarette smokers had a decreased risk of borderline statistical significance compared to nonsmokers (OR, 0.69; 95% CI, 0.47-1.01), particularly among premenopausal women (OR, 0.60; 95% CI, 0.38-0.96). This negative association persisted after adjustment for parity, hormonal treatments, and education. Women who started smoking before the age of 15 experienced a marked reduction in risk (OR, 0.38%; 95% CI, 0.18-0.80¿). Moreover, there was a suggestion of a dose-response effect with the amount of cigarettes smoked daily and with duration of the habit. Both the increased risk of artificial menopause and the negative association with smoking are compatible with a relation between levels of estrogens and thyroid cancer among women.

Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature.

Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.

The delimitation of tubular carcinoma of the breast.

Five hundred consecutive breast carcinomas from the first screening round of the Breast Cancer Detection Demonstration Project were studied quantitatively and semiquantitatively for features relevant to the diagnosis of tubular carcinoma. Tubularity was defined as the proportion of tumor cells that were adjacent to an open lumen. Nuclear morphology and mitotic activity were graded 1 to 3, and the presence of apocrine snouts as absent, few, common, or prominent. In plots and statistical cluster analysis, tubular carcinoma appears as part of a continuous spectrum of morphologies and not as a distinct entity. In multivariate analysis, apocrine snouts had no significant association with either nodal status or deaths of breast cancer. Tumors with 70% or greater tubularity by our definition and mitosis and nuclear grades 1 were not associated with either nodal metastases or deaths of breast cancer. The question is raised whether tubular carcinoma at the benign end of a spectrum shades into benign glandular proliferations, with particular reference to microglandular adenosis. A uniform and precise definition of tubularity is needed for the attainment of sufficient collective experience to delimit tubular carcinoma both from more aggressive carcinomas and from benign proliferations.

Observer variation in histologic classification of malignant and borderline ovarian tumors.

Eight hundred sixty-nine primary malignant or borderline ovarian tumors reported to the Norwegian Cancer Registry were reviewed. The histologic slides were randomly distributed to six observers and classified according to the World Health Organization classification of ovarian tumors. By rotation of slides, each tumor was successively reviewed by three observers. Each observer was given approximately 40 duplicates of slides he or she had typed before, mixed in with the slides for the third review. A contracted version of the classification with 27 entries was used in the analysis. Mean intraobserver reproducibility was 62% (kappa, 0.53), varying from 50% to 75% (kappa, 0.34 to 0.70) for the individual observers. The mean rate of agreement between two observers was 56% (kappa, 0.46), varying from 46% to 65% for the individual pairs of observers. The rate of full agreement among three observers was 41%. The most common disagreements were between different specific types of carcinoma, between undifferentiated and differentiated carcinoma, between borderline and malignant tumors, between unclassified and classified carcinoma, and between mixed and pure types of carcinoma. Very low reproducibility was obtained for mixed and unclassified carcinoma.

Remote frozen section service: a telepathology project in northern Norway.

We discuss the organization of a remote frozen section service in northern Norway. The service is operated by remote control of a motorized video-microscope located at Kirkenes Hospital, at a distance of more than 400 km from the workstation at the University Hospital in Tromsø. The video images of the frozen section are transmitted via a two-way telephone and video telenetwork with a 2 Mbit/s capacity. The images are displayed on monitors and diagnosed by pathologists in Tromsø. To date, 17 patients have been examined by remote frozen section. Correct benign versus malignant diagnoses have been given in all 17 cases compared with final diagnoses based on formalin-fixed and paraffin-embedded material. The average time taken for examining each frozen section was 15 minutes (range, 5 to 30 minutes). In none of the cases was the interpretation of the slides difficult due to deficient quality of the video images. For small hospitals with limited availability of local pathology services and for hospitals with a deficiency of specialists, telepathology may be a worthwhile substitute.

Cervical neoplasia in pap smears: risk of cervical intra-epithelial neoplasia (CIN) after negative or no prior smears in a population without a mass screening programme.

BACKGROUND: The aim of this study was to examine, in a population not submitted to mass screening, the risk of cancer and cervical intra-epithelial neoplasia (CIN) in Pap smears from women without previously reported positive smears. METHODS: In a logistic regression model consisting of 58,271 smears from 40,536 Norwegian women the risk of cytologically indicated CIN was studied according to age and time elapsed since last smear. RESULTS: The risk of CIN was highest in smears from women with no previously registered smears and in smears taken after an interval of > or = 5 years. Odds ratio for CIN I-II adjusted for age was highest in first time smears and in smears taken after an interval of 5 years. Odds ratio for CIN III was highest in first registered smears. No difference in risk of CIN III was found in smears taken within one year or 2-3 years after the last smear. The increased risk of CIN III in first smears was most pronounced in postmenopausal women ( > 50 years). Nine of 16 cases with cytological indication of cancer were found in women having a smear taken for the first time. All cases with malignancy in smears were > 50 years. CONCLUSIONS: The risk of cytologically diagnosed premalignant cervical conditions increases with time since the previous smear, but more than 5 years have to elapse before the risk is comparable with that in first smears taken. Postmenopausal women without previous smears run the highest risk of serious cervical premalignancies and cancer.

Subclassification of Hodgkin's disease, nodular sclerosis type. Prognostic value?

Recently, studies have suggested a subclassification of Hodgkin's disease, nodular sclerosis (HDNS). Between 1985-1994, twenty-four patients with HDNS were treated at the University Hospital of Tromsø, Norway. The cases were subclassified according to the histological criteria from the British National Lymphoma Investigation (BNLI) into NS1 (15 patients) and NS2 (9 patients). The relative frequencies of the two subtypes of HDNS in our region were in agreement with other reports. After a median follow up of 33 months, no significant difference between NS1 and NS2 with regard to complete remission rate (87% versus 100%) or predicted 5-year actuarial survival (93% versus 75%) were observed. Although our data are too small to draw specific conclusions, the discrepancy with some of the previous studies could be due to an improved response of the more aggressive type (NS2) to optimal treatment. We hope this study may initiate a raised interest in this field.

[Quality assurance in a department of pathology and anatomy]. / Kvalitetssikring i en patologisk-anatomisk avdeling.

The author describes a quality assurance programme in a department of anatomic pathology. An internal quality assurance committee consisting of four consultants, two chief technicians and a chief secretary meets monthly to decide what measures should be carried out each month. The main activities have been to review random cases of surgical pathology, frozen sections, diagnostic revisions, turn-around times and single event reports. Problems of diagnosis are discussed at a medical staff meeting before they are put before the committee. The programme has been well received in the department.

Renal endometriosis. A case report.

In a 23-year-old woman with lumbar pain, an expansive process in the right kidney was demonstrated by I.V.P., retrograde pyelography and renal angiography. The histological examination subsequent to nephrectomy showed endometriosis in association with localized xanthogranulomatous pyelonephritis. Renal endometriosis has been described only eight times previously in the literature. The current case is discussed and compared with previous reports.

Basal cell adenomatosis of minor salivary glands of the upper lip.

The basal cell adenoma is one of the more commonly occurring types of salivary tumors in the upper lip. A case with multiple (five) tumors is presented and the literature reviewed. This lesion would be more correctly designated as an adenomatosis. In spite of its frequent multicentricity, recurrence is unusual following surgical removal.

[A snow avalanche in Vassdalen. Medical experiences]. / Skredulykken i Vassdalen. Medisinske erfaringer.

Of 31 soldiers caught by an avalanche in Northern Norway, one was not buried, 13 were partly buried and 17 were completely buried by the mass of snow. Only one of the completely buried soldiers survived. On admission to hospital after being buried by snow for three hours he had moderate hypothermia. The cause of death in the other 16 was most probably general compression of the body with acute respiratory and circulatory failure. Five of the 13 partly buried had physical traumas. None of the nine attempts at resuscitation succeeded. Self-rescue and rescue by friends were most important to the soldiers who survived. We discuss aspects of the organized rescue operation.

[Cervical intraepithelial neoplasms. Persistency and recurrence after laser surgery]. / Cervikal intraepitelial neoplasi. Persistens og residiv etter laserbehandling.

Between 1983 and 1988, 402 women, with ages ranging from 18 to 73 years were treated for cervical intraepithelial neoplasia using the CO2-laser. 89 patients underwent vaporization and 313 conization. Preoperative biopsy examination showed CIN III in 214 patients. Five cases of invasive cancer were diagnosed, either preoperatively (two cases), after conization (two cases) or on both occasions (one case). At the first postoperative examination residual disease was found in 21 patients of the vaporization group (23.6%) and in 13 patients of the conization group (4.3%). Later examinations (after 5-70 months) revealed recurrence in 10 patients in the vaporization group (11%) and in 13 patients in the conization group (4.3%). Median time for recurrence was 11 months. 30 patients were treated twice or more. No case of invasive cancer was diagnosed after treatment.

Polyps of the large intestine in Northern Norway.

In unselected autopsies on 171 males and 109 females over 20 years of age in Tromsø, Northern Norway, adenomas of the colon and rectum were present in 40% of the males and 33% of the females. The frequency of adenomas increased with age in both sexes. Hyperplastic polyps were found in 20% of all cases with no variation in frequency with sex or age. Before the age of 65 most adenomas were located in the distal half of the large intestine in both sexes. After 65 years in males and 75 years in females the predilection site was shifted to the proximal half of the large intestine. The average size and grade of atypia of adenomas increased with age, but no significant difference in grade of atypia was found between the proximal and distal halves of the large intestine. The occurrence of adenomas was not associated with any of the common chronic diseases thought to be related to a western-style diet or to malignant or benign neoplasms elsewhere in the body. A significant association was found between the occurrence of adenoma and high body weight. The findings support the role of adenomas as precursors of colorectal carcinoma.

Diverticular disease of the large intestine in Northern Norway.

In 280 unselected necropsies on patients over 20 years of age in Northern Norway, diverticular disease was present in 25% of the males and 43% of the females. The frequency of diverticular disease increased in both sexes by age. Both the frequency of diverticular disease and the average number of diverticula per case with diverticular disease were higher in females than in males in all age groups. The sigmoid was the most frequent site of diverticula in both sexes and for all ages, and the average number of diverticula per diverticulum-bearing segment was also highest in the sigmoid for all ages and in both sexes. The average number of diverticula in the sigmoid of affected individuals increased with age and with the number of segments involved. Diverticular disease was not associated with adenomas of the large intestine or with malignant or benign neoplasms elsewhere in the body or with any of the common diseases thought to be related to a Western type of diet, except with cerebrovascular disease.

Topographic criteria in the diagnosis of tumor emboli in intramammary lymphatics.

Topographic relationships to adjacent structures were used as criteria to identify intramammary lymphatics with tumor emboli in breast cancer patients, in addition to conventional morphologic criteria. Patterns of relationship to blood vessels, non-neoplastic lobules and ducts, and empty lymphatics were defined. Ninety-five cases were independently reviewed by two observers. Interobserver reproducibility of the diagnosis of lymphatic vessel invasion (LVI) was 82% (kappa 0.60). The observers agreed on the presence of LVI in 23 patients (24%), of whom 21 (91%) had positive lymph nodes. Only among patients in whom more than ten emboli were identified was the frequency of positive lymph nodes markedly higher than in the total material. The location of tumor emboli relative to the invasive tumor was of little significance. LVI was a more powerful predictor of lymph node status than tumor size, margin contour, histologic grade and histologic type, and was highly significant also when controlled for these features.