Primary leiomyosarcoma of the breast: diagnosis, management and outcome. A report of a new case and review of literature.

Primary leiomyosarcoma of the breast is very rare and represents a diagnostic challenge. Only 16 cases have been reported in the English language literature. We report another new case and have analysed reports of the previous cases aiming to present a simple evidence-based approach to the clinical, radiological and pathological diagnosis of this rare tumour. The average age of presentation is 56 years. All neoplasms have been limited to the breast at the time of diagnosis. The usual presentation is a slowly growing mass, but in the current case report the patient present with mastalgia. There is always a possibility of local recurrence or distant spread, which can occur many years after primary surgery. Leiomyosarcoma must be histologically distinguished from leiomyoma and the presence of >3 mitoses per 10 high-power fields is usually indicative of malignancy. Immunohistochemistry is helpful to confirm diagnosis. As this is a slow-growing malignant tumour with a propensity for local recurrence, total mastectomy is the treatment of choice. The tumour is not hormone-dependent and hormone manipulation is not a treatment option.

Can thymidine kinase levels in breast tumors predict disease recurrence?

BACKGROUND: Our previous study of thymidine kinase (TK) levels in the serum of breast cancer patients demonstrated a statistically significant positive correlation with cancer stage. In postsurgical follow-up studies of 20 patients with primary breast cancer, total serum TK levels rose with disease recurrence and continued to rise with disease progression but decreased with treatment response. PURPOSE: This study was designed to examine whether TK levels in primary breast tumors can be used to predict recurrence and to establish the relationship between TK levels and estrogen receptor (ER) status and recurrence. METHODS: Eighty-six patients with breast cancer were entered in this study. Tumors were assessed for ER status and TK levels, and the patients had follow-up for recurrence over a period of 41 months. By calculating the percent of TK activity in the presence of adenosine triphosphate (ATP) or cytidine triphosphate (CTP), we estimated the relative contributions of TK isozymes TK1 and TK2 to total TK activity. RESULTS: Total TK (TK1 plus TK2) levels in tumors were significantly (P < .001) elevated in patients who subsequently had recurrence compared with levels in those who did not. Calculations of the percent of TK activity in the presence of ATP or CTP showed that this elevation was due to increased TK1 isozyme levels. ER-negative (ER-) patients had significantly (P < .001) increased TK1 levels relative to those in ER-positive (ER+) patients. ER- patients with recurrence had significantly (P < .001) elevated total tumor TK levels compared with levels in those who did not have recurrence, and calculation of percent of TK activity with ATP or CTP indicated elevated TK1 levels. A similar pattern of increased levels of total tumor TK and TK1 was observed in ER+ patients with recurrence. CONCLUSIONS: The results indicate that total tumor TK levels were significantly higher in breast cancer patients who subsequently had recurrence than levels in those who did not. This finding appears to be largely caused by higher levels of TK1. IMPLICATIONS: Higher TK1 levels in tumors in patients who subsequently had disease recurrence almost certainly indicate a high rate of proliferation in such tumors at the time of excision. It appears that TK is a potentially useful marker in the management of breast cancer. With measurement of levels of TK, particularly TK1, in breast tumors and serum, it may be possible to predict recurrence of breast cancer.

Breast tumour thymidine kinase levels and disease recurrence.

Thymidine kinase (TK) exists in two forms, TK1 and TK2. TK levels and oestrogen receptor status (OR) were measured in tumours from 86 patients with operable breast cancer and the patients were monitored for recurrence over 24 months. During the monitored period, 13 patients showed recurrence. These patients also exhibited higher total TK levels per mg tumour (P < 0.01) and higher TK1 levels (P < 0.001) than those who did not show recurrence. TK1 levels relative to TK2 were significantly higher (P < 0.05) in OR-negative tumours (n = 29) than in OR-positive tumours (n = 57). OR-negative (n = 9) and OR-positive (n = 4) patients who recurred had significantly higher TK1 levels relative to TK2 than did those who not recur (n = 20 and n = 53, respectively). These preliminary results indicate that breast tumour TK levels may have value in determining prognosis.

An evaluation of radionuclide bone scanning and liver ultrasonography for staging breast cancer.

The use of nuclear bone scanning and liver ultrasonography to stage breast cancer is an established practice in many hospitals. A 3 year prospective study was undertaken to assess the usefulness of these two investigations. Three hundred and fifty-eight patients were analysed: 133 had stage I disease, 188 were stage II and 37 were stage III. Bone scans were performed on 339 (94.7%) patients; 302 had stage I or stage II disease; and 37 were stage III. Bone scans were positive for metastases in only 0.9% of stage I and II patients but were positive in 16.2% of patients with stage III disease. None of the 309 (96.2%) stage I or stage II patients who had an ultrasound scan had any liver metastases detected whereas positive scans were obtained in 5.4% of stage III patients. It can be concluded that the incidence of demonstrable bone or liver metastases in stage I and stage II breast cancer patients is so low that the use of routine scanning can be abandoned.

Wound sepsis in 10,000 surgical patients.

A twelve year prospective wound audit was undertaken in an academic surgical unit. Data from 10,000 operations were analysed. Overall, wound infection rates decreased during this time. Infection rates in contaminated wounds in particular fell from 19.2% to 4.7%. This decrease in wound infection may be related in part to a change in the antibiotic prophylactic regimen and in part to the institution of the wound sepsis audit which provided regular information on the unit infection rates. This audit permitted early detection of adverse trends, and may have had a direct influence on surgical techniques.

Determination of bile acids and their conjugates in serum by HPLC using immobilized 3 alpha-hydroxysteroid dehydrogenase for detection.

A method is described for the separation of unconjugated bile acids and their glycine and taurine conjugates in a single step with adequate sensitivity for analysis of serum samples. Separation was carried out over a period of 80 min using a linear gradient with increasing concentrations of methanol in aqueous ammonium dihydrogen phosphate buffer, on an ODS Spherisorb column. Spectrofluorometric detection of NADH, formed as the column eluate passed through a column of immobilized 3 alpha-hydroxysteroid dehydrogenase, enabled amounts less than 40 pmol to be quantified. The reaction was carried out at neutral pH so that the lifetime of the enzyme column was increased, compared to other methods where a pH nearer 9.0 is commonly used. Flow rates were optimized to give comparable peak area for both primary and secondary bile acids.

Bleeding oesophageal varices with long term follow up.

Complete long term follow up was obtained in 27 children who had bled from oesophageal varices. Most presented with haematemesis or melaena at an average age of 5.2 years in the portal vein thrombosis group (20 children) and 9.5 years in the intrahepatic group (7 children). All had splenomegaly. Only 6 of 20 children with portal vein thrombosis had a possible precipitating factor. A total of 182 admissions for bleeding are reported, in 68 of which injection sclerotherapy was used to control bleeding. Control rate with injection sclerotherapy was 97%. Shunts performed below age 10 years were associated with a high thrombosis rate. A conservative approach to bleeding varices in children is recommended with transfusion, pitressin, and injection sclerotherapy. Oesophageal transection may have a role in the emergency management of the few children in whom bleeding is not controlled by injection sclerotherapy.

Idiopathic intestinal pseudo-obstruction: a familial visceral neuropathy.

Four individuals with idiopathic intestinal pseudo-obstruction (IIP), a familial visceral neuropathy with autosomal dominant inheritance, are presented. The disorder is characterised by abdominal colic, abdominal distension and diarrhoea, and is of a progressive nature with relapses and remissions. The main feature is a disturbance of motility in the affected intestine, with histological evidence of a neuronal disorder, characterised by hyperplasia and eventual atrophy. Management of the condition is difficult; initially, treatment should be conservative by nasogastric suction with intravenous nutrition. When laparotomy is undertaken to exclude mechanical obstruction, resection of the intestine is inadvisable, and ileo-colic anastomosis may be beneficial.