[Arrhythomgenic right ventricular dysplasia and sudden death: An autopsy and histological study]. / Dysplasie arythmogène du ventricule droit et mort subite : étude autopsique et histologique.

Arrhythmogenic right ventricular dysplasia (ARVD) is cardiomyopathy where normal myocardial tissue is replaced with fibrofatty tissue. Histological examination performed on myocardial biopsy or on autopsy samples are used to confirm the diagnosis. However, in many cases, the diagnosis cannot be made on a simple macroscopic and histological study and requires genetic analysis and molecular biology. In this work, we propose to describe the main macroscopic and histological findings of ARVD through the study of an autopsy series. We report 12 autopsy cases of sudden death in ARVD collected in the Department of Forensic Medicine of the University Hospital Fattouma Bourguiba Monastir (Tunisia) during a period of 20years. Microscopic examination was performed on 5microns thick histological sections. All slides were reviewed by two operators in a double blind (physician pathologist, pathologist) and in each, the percentage of adipose tissue, fibrosis and infarction in the right ventricle, left ventricle and interventricular septum, the presence or absence of inflammatory infiltrate, the presence or absence of signs of degeneration of myocytes were noticed. ARVD was found in 12 cases (1.8% of sudden cardiac death). The age ranged between 13 and 67years (mean age: 45.3years). The death occurred in half of the cases during exercise. Macroscopic examination of the RV showed the presence of a wall thinning (thickness<3mm) in 9 cases. Histological study highlight RV adipose infiltration in all cases with a percentage between 15% and 60%, fibrotic lesions were observed in only 9 cases with an average percentage of 10.25% and signs of degeneration of myocytes were noted in 10 cases. In concordance with what has been reported in the literature, there is still no consensus regarding the criteria to be adopted to pose with certainty the diagnosis of ARVD and the presence of adipose tissue remains the criterion more suggestive.

Posterior interosseous nerve syndrome due to intramuscular lipoma.

Lipomas are extremely common benign soft tissue tumors that are usually subcutaneous and asymptomatic. However, an intramuscular lipoma, occurring adjacent to the proximal radius, may easily cause paralysis of the posterior interosseous nerve because of a specific anatomical relationship of these structures in that area. In this report, we describe an unusual case of a 48-year-old-woman with a posterior interosseous nerve syndrome due to an intramuscular lipoma. The patient had good recovery after surgery and rehabilitation physiotherapy.

Rhino-orbito-cerebral mucormycosis: five cases.

INTRODUCTION AND OBJECTIVE: Mucormycosis is an invasive fungal infection usually observed in immunocompromised patients. Mucormycosis is rapidly fatal without an early diagnosis and treatment. We report five patients of rhino-orbital-cerebral mucormycosis and a literature review. DESIGN: The medical records of five patients presenting with rhino-orbital-cerebral mucormycosis, admitted between January 1995 and December 2007, were analyzed. All patients underwent tissue biopsy. The histologic sections revealed the presence of non-septate hyphae of the order Mucorales. RESULTS: The five patients, three men and two women, between 27 and 61 years of age, were all diabetic. The main symptoms were exophthalmia (five patients), facial swelling (four patients), periorbital cellulitis (four patients), and cranial nerve palsy (four patients). Anterior rhinoscopy revealed palatine or nasal necrotic lesions in four patients. All presented with diabetic ketoacidosis and CT scan revealed rhino-orbital-cerebral involvement in every patient. All patients were given intravenous amphotericin B. Four patients underwent surgical debridement of necrotic tissue. Two patients survived. CONCLUSIONS: Mucormycosis is usually a fatal infection in diabetic patients. Early diagnosis should be based on imaging data and histology. Amphotericin B must be rapidly initiated and associated with aggressive surgical debridement to reduce mortality.

Small cell carcinoma of the ovary of hypercalcemic type: a case report.

Introduction. The small cell carcinoma of hypercalcemic type of ovary is a very aggressive tumor. It is associated with two-thirds of cases with hypercalcemia most often asymptomatic. It occurs mostly for young women. The treatment combines surgery, chemotherapy, and radiotherapy. Case Presentation. We report a case of small cell carcinoma of the ovary hypercalcemic type in a young Tunisian woman aged 25 years after a severe abdominal pain syndrome and a large ovarian mass discovered in scanner; a laparotomy was performed by radical surgery. The pathological examination of the specimen confirmed the diagnosis. The radiological assessment performed after surgery showed a continuing evolution. Palliative chemotherapy was established, and the patient had died two months after diagnosis. Conclusion. The hypercalcemic small cell carcinoma of the ovary is a rare disease of poor prognosis.

Benign osteoblastoma in an unusual mastoid location.

INTRODUCTION: Benign osteoblastoma (OB) is an unusual primary bone tumor. The preferred locations are the posterior arch of vertebrae and long bones. We report herein an extremely rare location of an OB in the mastoid process of the temporal bone. CASE REPORT: A 22-year-old woman presented with painful left retro-auricular swelling. Computed tomography features were suggestive of an aggressive osteolytic lesion of the left mastoid. The pathologic examination of bone curettage material revealed a benign OB. A complete resection of the tumor was performed later, with no evidence of recurrence at 1 year. DISCUSSION/CONCLUSION: To our knowledge, this is the 14th reported case of OB confined to the mastoid process of temporal bone. Its histological diagnosis can be difficult and osteosarcoma is its principal differential diagnosis. Although generally regarded as benign, OB has potential for recurrence and local invasion. As such, complete resection, whenever possible, is preferred over conventional curettage.