Leiomyoma of the nasal cavity. A case report

Most of the sinonasal tumours are of epithelial origin. Smooth muscle tumours are extremely rare, they account for less than 2.5% of the mesenchymal neoplasms of the sinonasal tract and the nasopharynx. Fewer than 30 cases of primary leiomyogenic tumours have been reported in the sinonasal tract with almost an equal frequency of benign and malignant types. We report a case of a rare leiomyoma of the nasal cavity. The pathological and the clinical characteristics of this tumour are discussed.     

Acinar cell carcinoma of the pancreas: a rare tumor with a particular clinical and paraclinical presentation

Acinar cell carcinoma (ACC) of the pancreas is a rare tumor with an extremely low incidence rate. While the number of reported patients with ACC is relatively small, a long-term survival rate has been noted in patients with neuroendocrine differentiation. A 39-year-old woman visited our emergency department for upper gastrointestinal bleeding. Endoscopy indicated extrinsic compression of the posterior body of the stomach, together with a large, 10-cm, central ulcer covered with necrotic tissue. Abdominal computed tomography (CT) indicated a lesion that involved the whole of the pancreas, with a fistula in the stomach, which was suspected of being a degenerative intraductal papillary mucinous tumor of the pancreas. Magnetic resonance imaging (MRI) of the pancreas was performed, and the results further strengthened our suspicions by demonstrating the presence of cystic lesions and tumor buds. A total duodenopancreatectomy, including total splenectomy and gastrectomy, was performed, along with two independent Roux-en-Y anastomoses (one esojejunal and one hepaticojejunal). The tumor also had a wide opening in the stomach. The patient's postoperative course was marked by partial thrombosis of the portal vein, which was treated medically. Histopathological examination provided evidence of pancreatic ACC. The diagnosis of ACC should be considered in the presence of cutaneous lesions, which were absent in the case of our patient, and colonoscopy is also highly desirable because of the various forms associated with familial adenomatous polyposis. The prognosis, which includes a 5-year survival rate of 45%, in the population with an R0 resection is better than that for ductular adenocarcinoma, thus prompting the more aggressive management of this type of tumor.     

Primary hydatidosis of the central nervous system: A retrospective study of 39 Tunisian cases

Objective

To analyze epidemiological characteristics, clinical symptoms, radiological aspects, treatment and outcome of central nervous system hydatidosis and compare our results with those reported in literature.

Patients and methods

In our retrospective study, we reviewed 39 cases of primary central nervous system hydatid cysts operated on in our hospital between 1998 and 2007.

Results

There were 20 male and 19 female patients (sex-ratio M/F = 1.05) between 2 and 68 years of age (mean = 26.5 years). Thirteen of the patients were children (33.3%) with a mean age of 6.8 years and 26 were adults (66.7%) with a mean age of 36.3 years. The location of hydatid cysts was intracranial in 27 cases (69.2%) and spinal in 12 cases (30.8%). Headache and motor deficits were the predominant symptoms in patients with intracranial hydatidosis whereas back pain and spinal cord compression syndrome were the most frequent clinical presentations in patients with spinal hydatidosis. All patients underwent surgical resection of the cyst. Pathologic findings were consistent with hydatid cyst in all cases. During the follow-up period which ranged between 12 months and 5 years, 12 patients had recurrence (30.7%). Only one patient with intracranial hydatid cyst died postoperatively due to anaphylactic shock.

Conclusion

Despite all the advances in imaging techniques and therapeutic methods, central nervous system hydatidosis remains difficult to cure and patient outcomes are not satisfactory especially in case of spinal involvement due to the high incidence of recurrence.     

Rheumatologic manifestations of sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It has various clinical features. The most commonly affected organs are the lung, the lymph nodes, the eyes and the skin. Involvement of the musculoskeletal system is far less common and may be inaugural. Articular involvement is dominated by Lofgren syndrome and acute polyarthritis. Abarticular manifestations are often confounded with arthritis. Bone locations are dominated by sarcoidosis dactylitis and osteolysis. Muscular involvement is often unknown and can appear as 3 clinical features: spread form, myositique form or pseudotumoral form. Calcium balance disturbances are dominated by hypercalcemia which is often asymptomatique, but sometimes it reveal the sarcoidosis. Treatment of rheumatologic disorders often involves non steroidal antiinflammatory drugs, corticosteroids and methotrexate. Biological therapies such as the anti-TNFa and the anti-CD20 were showed to be effective in some case reports of severe and refractory disease.