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.

[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.

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.

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.

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.

Multiple paraneoplastic syndromes revealing non-small cell lung carcinoma.

A broad spectrum of paraneoplastic dermatoses is associated with lung cancer. We report herein a 56-year-old man who presented an association of erythroderma, acquired ichthyosis, palmo-plantar keratoderma, hypereosinophilia and hyper IgE. In light of these clinical and biological assessments an underlying malignancy had been suspected. A thoracic, abdominal and pelvic computed tomography showed a left hilar mass. The patient underwent a left pneumonectomy and the histological study had confirmed a non-small cell lung cancer. Recognition of cutaneous paraneoplastic syndromes is important since it leads to prompt diagnosis of an underlying malignancy and consequently a better management and prognosis of the disease.

Cyclophosphamide-induced generalised reticulated skin pigmentation: a rare presentation.

CASE: We describe a 55-year-old woman suffering from Sezary syndrome, had undergone chemotherapy consisting of cycles of cyclophosphamide and prednisone. 10 months later, she noticed a progressively increasing reticulated generalised pigmentation in the face, trunk and the extremities. Cylophosphamide was withdrawn. The hyperpigmentation began to clear slowly and gradually after 7 months. One year after cyclophosphamide withdrawal and facing the relapse of the disease, and its transformation to a large T cell lymphoma a mini CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) protocol was held, leading to a remarkable aggravation of the pigmentation. CONCLUSION: This adverse drug reaction to cyclophosphamide is peculiar by its localization and distribution and should be known in order not to confuse with other dermatosis.

[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.

Merkel cell carcinoma with lymphoepithelioma-like pattern: a case report of an exceedingly rare variant of merkel cell carcinoma with lymph node metastases at presentation.

Merkel cell carcinoma (MCC) or primary neuroendocrine carcinoma of the skin is a rare neoplasm with aggressive behavior. Primary lymphoepithelioma-like (LEL) carcinoma of the skin is a recently described exceptional tumor, with a relatively good prognosis, and is characterized by a neoplastic epithelial component associated with a dense lymphoid stroma. Rarely, MCC shows a marked lymphocytic host response or can even mimic a LEL carcinoma. We report a new case of MCC mimicking an LEL carcinoma in a 72-year-old male; the diagnosis of MCC was made on the basis of the morphology and immunohistochemical findings. We present through this case an exceptional pattern of MCC which can be misleading, and we insist on differential diagnoses.

Bilateral ovarian serous borderline tumor with a giant non-invasive peritoneal implant in a four-year-old girl.

Epithelial ovarian tumors are uncommon before 20 years of age and rarely occur before puberty. The vast majority of these tumors are benign, and few cases of malignant and borderline tumors are described. We report a case of a 4-year-old premenarchal girl, previously healthy, who presented with two abdominal masses. Laboratory analysis of blood showed elevation of the serum level of the CA-125. Ultrasonographic examination disclosed bilateral cystic ovarian masses. Laparoscopic exploration revealed bilateral ovarian multicystic masses with retro-uterine peritoneal implant. Bilateral salpingo-oophorectomy with implant resection was performed. Histologic findings were consistent with a serous borderline tumors of both ovaries and the peritoneal implant was of the non-invasive type. There is no evidence of recurrence at 3-year follow-up. To our knowledge, there are only four cases of ovarian borderline tumors in premenarchal girls reported in the English literature: three of the mucinous type and only one of the serous type.

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.