Abdominal wall desmoid tumours.

We present two cases of desmoid tumour of the anterior abdominal wall in young women in whom the defect after radical excision could not be closed without using prosthesis. The first case warranted the use of a composite mesh, the second a polypropylene prosthesis. In both cases primary closure of the skin was possible. Both women are doing fine with no sign of relapse or incisional hernia.

Boerhaave's Syndrome: Still a Diagnostic and Therapeutic Challenge in the 21st Century.

Boerhaave's syndrome is a rare but potentially fatal condition characterised by a transmural tear of the distal oesophagus induced by a sudden increase in pressure. Diagnosis is challenging as the classic triad of vomiting, abdominal or chest pain, and subcutaneous emphysema is absent in many patients. Management is multidisciplinary and relies on rapid, distinct, and repeated imaging. Treatment has not been standardised and may be conservative, endoscopic, or surgical. We present a typical case which illustrates possible diagnostic pitfalls and the therapeutic conundrum surrounding management of the syndrome. Based on time of presentation and eventual presence of sepsis, a therapeutic algorithm is proposed.

Deep venous thrombosis and pulmonary embolism caused by an intravascular synovial sarcoma of the common femoral vein.

Malignant tumors arising in deep veins of the lower extremities are very uncommon. To our best knowledge, this is the seventh case of a primary venous intravascular synovial sarcoma (SS) reported in literature. A 32-year-old woman was admitted with a second episode of deep venous thrombosis of the right lower limb and pulmonary embolism. Physical and radiological examinations showed besides the thrombosis a tumor arising from the right common femoral vein involving the bifurcation of the common femoral artery. At surgery, en block resection of the tumor including the deep femoral vein and arterial bifurcation was done with an arterial reconstruction using a synthetic graft. Histopathological examination revealed an intravascular SS of the common femoral vein. The mainstay of curative therapy is complete surgical resection of all tumor manifestations with negative histological margins.

An incident case of primary acquired pulmonary alveolar proteinosis.

This is a case of a primary acquired pulmonary alveolar proteinosis (PAP) in an asymptomatic patient, on the waiting list for kidney transplantation, confirmed on lung biopsy and by identifying anti-GM-CSF antibodies.

Role of intra-operative touch imprint cytology in the treatment of breast cancer.

OBJECTIVES: A prospective study was carried out to evaluate the role of intra-operative touch imprint cytology (TIC) in the assessment of sentinel lymph node (SLN) involvement for staging and treatment of early-stage, clinically node-negative breast carcinoma. METHODS: Forty-five patients with early-stage, clinically node-negative breast cancer underwent a SLN biopsy with intra-operative TIC. The SLN was bisected if its width was less than 4 mm or sliced every 2 mm if it was more than 4 mm. The imprint specimens were stained with haematoxylin and eosin (H&E). Rapid immunochemistry (IH) was performed in case of equivocal cytological result. Permanent sections were evaluated with H&E and IH staining. The results of TIC were compared to histopathological results. RESULTS: The sensitivity, specificity and overall accuracy of TIC on a node basis were 65.5%, 96.3%, 85.5%, respectively. When calculated according to the size of SLN metastasis, the sensitivity of TIC for overt metastasis was 84.6%, while it was 62.5% for micrometastasis and 37.5% for sub-micrometastasis. The mean size of nodal metastasis was 5.08 mm and 1.25 mm for true positive and false negative results, respectively (P = 0.0236). Because of intra-operative TIC, 76.5% of the patients who needed further axillary lymph node dissection (ALND) could undergo this during the same operating time. CONCLUSIONS: TIC is a rapid and reliable method for the intra-operative assessment of metastatic sentinel node involvement in patients with early-stage, clinically node-negative breast carcinoma. Despite a low sensitivity comparable to frozen section (FS) in detecting micro- and sub-micrometastases, the technique offers the advantage of full tissue preservation for subsequent histological analysis.

Occurrence of pulmonary artery aneurysms and pulmonary artery thrombosis in a young man.

Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown aetiology. Arterial involvement is uncommon but associated with important morbidity and mortality. We describe the clinical course of BD with severe pulmonary artery involvement in a 19-year-old man. He presented with massive haemoptysis related to pulmonary artery aneurysms. Initial treatment consisted in urgent right inferior lobectomy, corticosteroids and monthly intravenous cyclophosphamide. Subsequently, he developed pulmonary artery thrombosis at non-aneurysmatic sites. Corticosteroid therapy was intensified, monthly intravenous cyclophosphamide was continued and an anticoagulant was added to the treatment with a favourable clinical and radiological response. Our case illustrates that timely initiation of highly potent immunosuppressive therapy is critical to obtain a favourable outcome. At present, a consensus regarding optimal management of vascular BD is lacking. ALthough anticoagulation is not generally recommended, our report encourages a patient-based decision after carefully tailoring potential risks and benefits.

Primary primitive neuro-ectodermal tumour of the lung.

A case of intrapulmonary primitive neuro-ectodermal tumour (PNET) without thoracic wall involvement is presented in a 33-year-old man. PNET of the thoracopulmonary region, also called Askin tumour, is a rare undifferentiated sarcoma usually involving the thoracic wall. Primary intrapulmonary PNET without parietal pleura or thoracic wall involvement is very rare. The correlation between anatomo-pathological aspects and clinical imaging is emphasized, which is discussed in the light of the most recent literature.

Frey's syndrome after elective thyroidectomy: a case report.

This case report documents the case of a 41-year-old Caucasian woman who developed a Frey's syndrome after elective thyroidectomy. This patient developed a sudden redness on one side of the face in the recovery room after a total thyroidectomy under general anaesthesia. All other vital signs of the patient remained normal. There were no signs of infection. After a few hours the symptoms disappeared without any treatment. Frey's syndrome is a disorder characterised by unilateral flushing or sweating of the facial skin. This syndrome can occur after parotidectomy or after trauma, injury or inflammation of the parotid, the submandibular glands, or of cervical and thoracic parts of the sympathetic trunk. Frey's syndrome normally results from aberrant regeneration of auriculotemporal nerve fibers to sweat glands in the skin. This case describes a self-limiting Frey' s syndrome after irritation of the cervical portion of the truncus sympathicus following total thyroidectomy.

Tolerance of adjuvant letrozole outside of clinical trials.

Recently aromatase inhibitors have become a standard care as an adjuvant treatment for many postmenopausal patients with hormone receptor positive early breast cancer. Adjuvant letrozole was made available either immediately postoperative, after 2-3 years of tamoxifen, or as an extended treatment after 5 years of tamoxifen. Between October 2003 and October 2005, we analyzed the subjective tolerance in 185 postoperative early breast cancer patients receiving letrozole outside of a clinical trial. The most prominent toxicity was musculoskeletal pain. In addition hot flushes, increased fatigue, nausea, vomiting, anorexia, mood disturbances, vaginal dryness, hair loss and rash were also recorded. In contrast to the prospective randomized clinical trials, a high drop-out rate of 20% was documented, mainly due to aromatase inhibitor-associated arthralgia syndrome interfering significantly with the daily life of our patients. Although adjuvant aromatase inhibitors have proven to be generally superior to tamoxifen in the adjuvant setting, it is important to focus attention on the tolerance during the adjuvant therapy and to balance this against the potential benefit in individual patients. Alternative options including switching to tamoxifen remain available.

Granulomatous lobular mastitis: a rare chronic inflammatory disease of the breast which can mimic breast carcinoma.

Granulomatous lobular mastitis is a rare chronic inflammatory disease of the breast. The differential diagnosis with malign breast disease is often not easy. In most cases a surgical biopsy is needed for correct diagnosis. Idiopathic granulomatous mastitis is an exclusion diagnosis, based on the demonstration of a characteristic histological pattern, combined with the exclusion of other possible causes of granulomatous breast lesions. There is still no generally accepted optimal treatment. If surgery forms part of the treatment, a conservative approach seems to be adequate in most cases. Another option is a long-term steroid treatment. It is mandatory to exclude infectious causes of granulomatous mastitis before corticoid therapy is started.

Use of a Port-a-Cath system in the home setting for the treatment of symptomatic recurrent malignant pleural effusion.

Symptomatic malignant pleural effusions represent a common problem in metastatic cancers and are associated with a significant morbidity. Pleurodesis still remains the primary therapy of choice. In a few cases, however, pleurodesis is unsuccessful because of a limited lung expansion and pleuroperitoneal shunts have been used. We describe two cases where an implantable PORT-A-CATH system is used for regular drainage of the pleural effusion. The main advantage of this technique is the fact that the procedure of drainage can be performed by a nurse in the home setting.

A case of fatal necrotizing fasciitis after intramuscular administration of diclofenac.

Necrotizing fasciitis is a rapidly progressive soft tissue infection that involves subcutaneous fat and spreads along the fascial planes. This disease has a potentially fatal outcome if not recognized in early. Several cases have been reported of a possible association between the use of non-steroidal anti-inflammatory drugs (NSAIDs) and the development or aggravation of necrotizing fasciitis. This association is still a subject of controversy. In this article we present a case of fatal necrotizing fasciitis occurring in association with intramuscular injections of diclofenac in a patient who was admitted for the symptoms of a urinary stone. Our opinion is that the intramuscular injections caused a locally aseptic necrosis, which was secondarily invaded by. Since this incident, our policy is to avoid the use of intramuscular injections of diclofenac and other NSAIDs in cases of potentially infectious diseases.