Pediatric lichen planus pigmentosus possibly triggered by mercury dental amalgams.

Lichen planus pigmentosus is uncommon in childhood and its treatment is often challenging. We report a case of cutaneous lichen planus pigmentosus in a 10-year-old boy, without oral mucosal involvement, two months after an amalgam dental restoration. The diagnosis was based on the histopathological examination of a skin biopsy, the positive patch test to mercury, and the improvement after amalgam removal. Our case report suggests that metal allergy may play a role, and amalgam replacement may be followed by clinical improvement.

Liver Subcapsular Hematoma: A Rare Cause of Sudden Unexpected Death.

The spontaneous subcapsular hematoma of the liver is very rare. There are only a few reported cases in the literature.Most reported cases of liver hematoma often occur during pregnancy as part of the hemolysis, elevated liver enzymes, and low platelet count syndrome. The other causes may be due to amylosis, rupture of hepatocellular carcinoma, adenoma, focal nodular hyperplasia, hemorrhagic cyst, or hemopathy. Idiopathic spontaneous subscapular hematoma is a rare and often fatal condition.We report a case of a 43-year-old woman having Steinert disease who died because of a fatal spontaneous liver hemorrhage occurring without any traumatism. We did not find any apparent cause that could explain this hemorrhage even after a histological study of the liver.

Assessment of MYCN amplification status in Tunisian neuroblastoma: CISH and MLPA combining approach.

BACKGROUND: Neuroblastoma (NB) shows a complex combination of genetic aberrations. Some of them represent poor genetic prognosis factors that require specific and intensive chemotherapy. MYCN amplification consists of the major bad outcome prognostic factor, it is indeed frequently observed in aggressive neuroblastomas. To date different methods are used for MYCN status detection. OBJECTIVES: The primary aim of our study was to provide a critical assessment of MYCN status using 2 molecular techniques CISH and MLPA. We also focused on the correlation between neuroblastoma genetic markers and patient's clinical course among 15 Tunisian patients. METHODS: we developed a descriptive study that includes 15 pediatric Tunisian patients referred to our laboratory from 2004 to 2011. We reported the analysis of fresh and FFPE NB tumors tissues. RESULTS: No significant correlation was found between COG grade and patients overall survival. Assessment of NMYC gene copy number by kappa statistic test revealed high concordance between CISH and MLPA tests (kappa coefficient = 0.02). CONCLUSION: Despite misdiagnosing of MYCN status fewer than 5 copies, MLPA remains an effective molecular technique that enables a large panel of genomic aberrations screening. Thus combining CISH and MLPA is an effective molecular approach adopted in our laboratory. Our results allow pediatric oncologists to set up the first Neuroblastoma therapeutic strategy based on molecular markers in Tunisia.

Cutaneous involvement in multiple myeloma: a case report with an unusual location.

Multiple myeloma (MM) is a rare cancer. Cutaneous involvement is uncommon with fewer than 100 cases described in the literature. We report herein a patient with MM, subtype IgA kappa, with unusual clinical presentation including the lower lip.

[Epidemiological, clinical features, therapeutic results and evolution of gastrointestinal stromal tumour: about 25 cases]. / Caractéristiques épidémiologiques, cliniques, thérapeutiques et évolutives des tumeurs stromales digestives: à propos de 25 cas.

BACKGROUND: Gastrointestinal stromal tumors (GIST) are mesenchymal tumors occuring in the majority of cases in the stomach and small intestine, rarely in rectum, colon, esophagus or mesentery. They are derived from cells of cajal or their precursor, and are typically CD117/KIT + (95%), CD34 + (70%). AIMS: is to study the epidemiological, clinical, therapeutic and evolution of gastrointestinal stromal tumors. METHODS: retrospective study including all patients with the diagnosis of GIST supported in the department of gastroenterology and surgery in universital hospital of Monastir. RESULTS: 25 patients were included, 12 men and 13 women with an average age of 60.5 years. Digestive symptomatology was dominated by gastrointestinal bleeding (n = 12) and abdominal pain (n = 12). The tumor was discovered incidentally in two patients. The small intestine was the most common site of the tumor (n = 10), followed by the stomach in 9 patients, rectum in two patients, the colon (n = 1), the bulb of water (n = 1), duodenum (n = 1) and liver in a patient. The tumor size ranged from 0.8 to 24 cm. GIST was localized in 16 patients, in whom therapeutic care based mainly on surgery and optimal broad. It was metastatic in 9 patients, in whom treatment using imatinib as first-line in 4 of them with a good response in 3 patients and the possibility of R0 surgery in one patient, initial stabilization and then a secondary exhaust in a patient. The first surgery was necessary in 5 patients in complicated situation or if diagnostic doubt. CONCLUSION: The best characterization of GIST thanks to advances in cancer research has led to improved treatment of these tumors. Surgery is the standard treatment in localized forms. Imatinib is the standard treatment in metastatic GIST first line as well as adjuvant after surgery.

[Sacrum B cell-non-Hodgkin's lymphoma complicating a chronic viral hepatitis C related to a blood exposure: a case report]. / Lymphome B non hodgkinien du sacrum compliquant une hépatite virale C chronique liée à un accident d'exposition au sang : à propos d'une observation.

Frequency of the association between non-Hodgkin's lymphoma (NHL) and the hepatitis C virus (HCV) infection is variable according to previous studies. Besides, direct and/or indirect implication of the HCV infection in the development of NHL is probable but, its pathophysiological mechanisms remain unclear. In this report, we described the case of a 49-year-old patient with a B-cell NHL of the sacrum complicating a chronic HCV related to a blood exposure, and we report the recent data of this association.

Simultaneous occurrence of two squamous cell carcinomas within a nevus sebaceous of Jadassohn in an 11-year-old girl.

The development of a squamous cell carcinoma within a nevus sebaceous of Jadassohn is very rare. We report here for the first time, the simultaneous occurrence of two squamous cell carcinomas within a single nevus sebaceous of Jadassohn.

[Ischemic colitis as a first sign of primary antiphospholipid antibody syndrome. A case report]. / Colite ischémique révélant un syndrome des anti-phospholipides primitif. A propos d'un cas.

BACKGROUND: The primitif antiphospholpid antibody syndrome is a clinico-biologic entity characterized by the artério-venous thromboses and the presence of circulating antibodies against membranous phospholipids. The systemic demonstrations and in particular ulcerated and ischemic colitises are brought back unusually during this affection. AIM: Report a new case. CASE REPORT: We bring back one observations of ischemic colitis complicated of perforation revealing a primitif antiphospholpid antibody syndrome at a male patient aged of 45 and requiring the surgical intervention. CONCLUSION: This observation recall an exceptional étiology but often unrecognized of ischemic colitises.

[The importance of fine needle aspiration biopsy in the diagnosis of parotid tumors]. / Intérêt de la cytoponction à l'aiguille fine dans le diagnostic des tumeurs parotidiennes.

This study aimed to determine the diagnostic value of fine needle aspiration biopsy (FNAB) and of its contribution to the management of parotid tumors. We conducted a retrospective study of 47 patients who had undergone parotidectomy as well as preoperative fine needle aspiration biopsy. The study highlighted that eighty one percent of patients had a benign tumor while 19% of patients had a malignant tumor. The sensitivity and specificity of FNAB were 78% and 92% respectively. Parotid tumors were correctly classified as malignant or benign in 89% of cases, overall accuracy was 64.4%. FNAB is a reliable examination providing preoperative informations about the treatment plan and the postoperative course.

Fetal Rhabdomyomatous Nephroblastoma in a 31-Year-Old Woman: A Case Report.

Fetal rhabdomyomatous nephroblastoma (FRN) is a rare variant of Wilms tumor with distinct morphologic features and biologic behavior compared to conventional nephroblastoma. It mainly occurs in patients under 4 years. In adults, extremely rare cases of nephroblastoma were reported. Among these cases, none has been interested a FRN. We report an exceptional case of a 31-year-old woman diagnosed with FRN discovered incidentally, to illustrate clinical and histopathological characteristics of this entity.

[Angiomatoid tumor of the thyroid gland: primitive angiosarcoma or variant of anaplastic carcinoma?]. / Tumeur angiomatoïde de la thyroïde: angiosarcome primitif ou variante du carcinome anaplasique?

Thyroid gland angiomatoid tumors are an extremely aggressive neoplasms with varied histological patterns and features of endothelial differentiation. The histogenesis of thyroid angiomatoid tumors has been controversial for many years: these tumors may be either a variant of anaplastic carcinoma, or an angiosarcoma. We report a case of thyroid angiomatoid tumor in a 68-year-old woman. We also discuss, through a review of the literature, the pathologic criteria that could be used to distinguish between angiosarcoma and anaplastic carcinoma of the thyroid.

[Myxoid plexiform fibrohistiocytic tumor without multinucleated giant cells]. / Tumeur fibro-histiocytaire plexiforme myxoïde sans cellules géantes multinucléées.

Plexiform fibrohistiocytic tumour is a rare soft-tissue tumour of intermediate malignancy. It is more prevalent in children and in young adults. We report a case of this tumour presenting as a nontender swelling on the shoulder of an 8-year-old girl. Clinically, the mass was subcutaneous and measured 2 cm in greatest diameter. The histopathological examination revealed a mesenchymal dermic and subcutaneous tumour composed of confluent nodules. These nodules were made of aggregates of histiocyte-like cells and concentric fascicles of spindle cells. The stroma was myxoïd or collagenous. This case was particular by the lack of giant cells and the focally myxoid background. The epidemiologic, clinicopathologic features and outcome of this lesion are discussed and similar published cases are reviewed.

[An unusual testicular tumour: splenogonadal fusion]. / Une tumeur testiculaire inhabituelle: fusion splénogonadique.

Splenogonadal fusion is a rare congenital anomaly that is often discovered at operation or autopsy. The diagnosis is difficult, but could be based on preoperative scintigraphy and frozen section histological examination to avoid useless orchidectomy. It is a benign lesion, which must be distinguished from testicular tumour.

[Micropapillary bladder carcinoma]. / Carcinome micropapillaire de la vessie.

Micropapillary bladder carcinoma is a recently described, rare variant of urothelial carcinoma reputed for its poor prognosis. The authors report the case of a 59-year-old man with invasive bladder tumour presenting with haematuria. Histological examination of resection chips revealed the typical histological features of high nuclear grade micropapillary carcinoma with invasion of the musculosa and the presence of numerous endolymphatic emboli. The authors discuss the clinicopathological and pathogenic features, treatment and clinical course of this tumour.

Prognostic value of indoleamine 2,3-dioxygenase activity and expression in nasopharyngeal carcinoma.

Indoleamine 2,3-dioxygenase (IDO) is an enzyme with an immunosuppressive effect whose function is diverted by tumor cells to counteract immune cell functions, inducing immune escape of tumor cells. The aim of this study was to investigate the clinical significance of IDO in nasopharyngeal carcinoma (NPC). Compared to controls, NPC patients' plasma IDO activity was significantly higher, especially among patients with metastatic cancer (p=0.005). The immunohistochemical analysis revealed that high IDO expression was observed in 74% of NPC tissues and the epithelial IDO expression was inversely correlated to T-cell infiltration. Kaplan-Meier survival analysis showed that whatever the localization, intratumoral or stromal, patients with a high IDO expression and low T-cell infiltration have significantly lower survival rates. Moreover, in multivariate analysis, intratumoral and stromal IDO expression were found to be independent prognostic factors for disease-free survival (p=0.016; HR: 3.52) and overall survival (p=0.015; HR: 4.76) respectively. Our findings provide evidence that IDO is involved in tumor immune evasion of NPC, suggesting that it could be a relevant therapeutic target for NPC.

[Cardiac amyloidosis revealing multiple myeloma]. / Amylose cardiaque révélatrice d'un myélome multiple.

INTRODUCTION: Amyloidosis is a rare disease characterized by an extracellular accumulation of a protein polysaccharid complex (Amyloid). Cardiac involvement is considered as a major prognostic factor. OBSERVATIONS: We report the case of two women, hospitalized for heart failure. The diagnosis of cardiac amyloidosis was suggested by echocardiography: Left ventricular concentric hypertrophy and typical amyeloid infiltration with hyperechoic, shiny and granite-like aspect of the interventricular septum. The histological confirmation was obtained by gastric biopsy in the first case and biopsy of the salivary glands in the second revealing an amyloidosis AL. This cardiac amyloidosis was secondary to multiple myeloma: monoclonal Gammopathy with immunoglobulin Lambda in the first and Kappa in the second, and the presence of a plasmocyte infiltration in the sternal puncture. CONCLUSION: Amyloidosis is a rare pathology, the cardiac involvement is frequent in the type AL and can occur with or without clinical manifestations. Echocardiography should be systematic in patients with confirmed amyloidosis.