[Pneumomediastinum during a dermatomyositis: a rare entity]. / Pneumomédiastin au cours d'une dermatomyosite, une entité rare: à propos d'un cas.

Dermatomyositis is a connective characterized by inflammation of skeletal muscle with cutaneous manifestations. Their etiologies, still unknown, associate environmental and genetic factors. Among lung complications described, interstitial pneumopathies are common complications. Other complications are rarely reported as pneumomediastinum. We report a case of pneumomediastinum with aeric in massive skin dissection occurred in a patient with dermatomyositis. We will discuss the frequency, causes and pathophysiology of the disease according to the literature.

Giant cell hepatitis with autoimmune hemolytic anemia in a nine month old infant.

Giant cell hepatitis (GCH) with autoimmune hemolytic anemia is a rare entity, limited to young children, with an unknown pathogenesis. We report the case of 9-mo old who presented with fever, diarrhea and jaundice four days before hospitalization. Physical examination found pallor, jaundice and hepatosplenomegaly. The laboratory workup showed serum total bilirubin at 101 µmol/L, conjugated bilirubin at 84 µmol/L, hemolytic anemia, thrombocytopenia and immunoglobulin G (IgG) and anti-C3d positive direct Coombs' test. The antinuclear, anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies, antiendomisium antibodies were negative. Serological assays for viral hepatitis B and C, cytomegalovirus, herpes simplex and Epstein Barr virus were negative. The association of acute liver failure, Evan's syndrome, positive direct Coomb's test of mixed type (IgG and C3) and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH. The diagnosis was confirmed by a needle liver biopsy. The patient was treated by corticosteroids, immunomodulatory therapy and azathioprine but died with septicemia.

Phyllodes Tumors of the Breast: A Review of 26 Cases.

BACKGROUND: Phyllodes tumors of the breast are rare and locally aggressive neoplasms. Our study aimed to report the experience of the Farhat Hached Hospital (Sousse, Tunisia) acquired during a 7-year period and to give an additional review of the available literature. METHODS: The authors analyzed retrospectively clinical, radiological, histopathological and therapeutic features as well as outcome in a series of 26 cases diagnosed as phyllodes tumors of the breast at the Pathology Department of Farhat Hached Hospital, Sousse, Tunisia, from 2003 to 2009. The slides were reviewed in order to classify the tumors into benign, borderline and malignant on the basis of the criteria proposed by the World Health Organization. RESULTS: All the cases occurred in women. The analysis of this series showed the following characteristics: mean age at diagnosis was 40 years (19 - 66), tumor size was 1.5 - 40 cm (mean: 7.8 cm); the chief complaint was a mammary mass; the right breast was affected in 14 cases, the upper outer quadrant was the most commonly involved site (42.3%); surgical treatment was used in all cases, 21 patients (80.8%) were treated conservatively (13 benign, 6 borderline, and 2 malignant) and 5 (19.2%) by radical surgery (1 borderline and 4 malignant); seven patients underwent post-operative radiotherapy; in 14/19 cases (73.7%), a good correlation was observed between intraoperative frozen section analysis and definitive histology; the tumor was classed as benign in 13 cases (50%), borderline in 7 cases (27%) and malignant in 6 cases (23%); follow-up data was available in 22 cases; the rate of recurrence was 23% (1 benign, 3 borderline, and 2 malignant) after a mean follow-up of 13.6 months; all the recurrent tumors were initially treated by lumpectomy and were close to margin at the initial pathologic examination; the treatment of recurrences consisted of simple mastectomy in 5 cases, and local excision in one case; three patients developed metastases, one of whom after recurrence; three patients have died. CONCLUSIONS: This is a substantial single institution experience of a rare tumor. Phyllodes tumors of the breast have an unpredictable outcome, thus a wide local excision, with an adequate margin of normal breast tissue, is the preferred initial therapy.